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1. Grossman AB, Kelly P, Rockall A, Bhattacharya S, McNicol A, Barwick T: Cushing's syndrome caused by an occult source: difficulties in diagnosis and management. Nat Clin Pract Endocrinol Metab; 2006 Nov;2(11):642-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cushing's syndrome caused by an occult source: difficulties in diagnosis and management.
  • INVESTIGATIONS: Serum potassium and bicarbonate, circadian rhythm of cortisol, low-dose and high-dose dexamethasone suppression tests, plasma adrenocorticotropic hormone (ACTH), corticotropin releasing-hormone stimulation test, CT scan of the pituitary, plain chest radiology, CT scan of the chest and abdomen, trans-sphenoidal pituitary biopsy and histology, CT scan and MRI of the thorax, MRI of the pituitary, octreotide scintigraphy, gastroscopy, colonoscopy, gut peptides, tumor markers, urine 5-hydroxyl-indole-acetic acid, resection, histology, immunocytochemistry and in situ hybridization.
  • DIAGNOSIS: Occult ectopic ACTH syndrome from a presumed appendiceal neuroendocrine tumor.
  • The tumor was only identified some 20 years from initial presentation.
  • MANAGEMENT: Adrenolytic therapy before bilateral adrenalectomy to cure Cushing's syndrome, glucocorticoid and mineralocorticoid replacement therapy, and then repeated surveillance over 20 years to locate the ectopic source of ACTH.
  • [MeSH-major] ACTH Syndrome, Ectopic / complications. ACTH Syndrome, Ectopic / diagnosis. Cushing Syndrome / diagnosis. Cushing Syndrome / etiology
  • [MeSH-minor] Adrenalectomy. Adult. Appendiceal Neoplasms / diagnosis. Appendiceal Neoplasms / secretion. Appendiceal Neoplasms / surgery. Appendiceal Neoplasms / ultrasonography. Carcinoid Tumor / diagnosis. Carcinoid Tumor / secretion. Carcinoid Tumor / surgery. Carcinoid Tumor / ultrasonography. Female. Humans. Long-Term Care

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  • [ErratumIn] Nat Clin Pract Endocrinol Metab. 2007 Jan;3(1):69. Balwick, Tara [corrected to Barwick, Tara]
  • (PMID = 17082811.001).
  • [ISSN] 1745-8366
  • [Journal-full-title] Nature clinical practice. Endocrinology & metabolism
  • [ISO-abbreviation] Nat Clin Pract Endocrinol Metab
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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2. Kaya M, Kanmaz T, Boleken ME, Yücesan S: Appendix carcinoid found incidentally during excision of a choledochal cyst: report of a case. Surg Today; 2005;35(5):418-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Appendix carcinoid found incidentally during excision of a choledochal cyst: report of a case.
  • We report the case of a 13-year-old girl in whom an appendix carcinoid was found incidentally during excision of a choledochal cyst.
  • Although incidental carcinoid tumors of the extrahepatic bile ducts have been reported, to the best of our knowledge this is the first published case of a choledochal cyst associated with an appendix carcinoid.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / diagnosis. Choledochal Cyst / surgery

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  • (PMID = 15864427.001).
  • [ISSN] 0941-1291
  • [Journal-full-title] Surgery today
  • [ISO-abbreviation] Surg. Today
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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3. Velusamy A, Saw S, Gossage J, Bailey S, Schofield J: Combined adenocarcinoid and mucinous cystadenoma of the appendix: a case report. J Med Case Rep; 2009;3:28
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  • [Title] Combined adenocarcinoid and mucinous cystadenoma of the appendix: a case report.
  • INTRODUCTION: Adenocarcinoid of the appendix is a rare malignant tumour with features of both adenocarcinoma and carcinoid, showing both epithelial and endocrine differentiation.
  • Mucinous cystadenoma is the commonest of the benign neoplasms of the appendix, with an incidence of 0.6% in appendicectomy specimens.
  • We report a rare combination of these tumours and discuss the latest treatment options.
  • Laparoscopy revealed an adenocarcinoid of the appendix in combination with mucinous cystadenoma.
  • CONCLUSION: Adenocarcinoids account for 2% of primary appendiceal malignancies.
  • Most tumours are less than 2 cm in diameter and 20% of them metastasize to the ovaries.
  • Right hemicolectomy is generally advised if any of the following features are present: tumours greater than 2 cm, involvement of resection margins, greater than 2 mitoses/10 high-power fields on histology, extension of tumour beyond serosa.
  • Chemotherapy mostly with 5-Fluorouracil and Leucovorin is advised for remnant disease after surgery.

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  • (PMID = 19171048.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2647933
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4. Young RH: From Krukenberg to today: the ever present problems posed by metastatic tumors in the ovary. Part II. Adv Anat Pathol; 2007 May;14(3):149-77
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  • [Title] From Krukenberg to today: the ever present problems posed by metastatic tumors in the ovary. Part II.
  • This is the second of a two-part consideration of metastatic tumors to the ovary.
  • The first tumor discussed is gastric carcinoma of intestinal-type whose ovarian manifestations have been the subject of a recent paper which emphasized its differences from the Krukenberg tumor.
  • The section on pancreatic neoplasms reemphasizes the problems caused by metastatic ductal carcinoma, considered primarily in Part I, and discusses less common issues such as spread of neuroendocrine and acinar cell carcinomas.
  • The limited information on spread of tumors of the gallbladder and extrahepatic bile ducts is then reviewed before more detailed consideration of hepatic neoplasms, prompted by recent contributions on hepatocellular carcinoma and intrahepatic cholangiocarcinoma, the latter based on significant experience with this problem in Thailand.
  • The section on appendiceal neoplasms highlights ovarian spread of diverse tumors ranging from typical intestinal-type adenocarcinoma to signet-ring cell carcinomas with various patterns which in the ovary may prompt diagnoses such as a goblet cell (mucinous) carcinoid tumor, but whose ovarian features place them in the category of a Krukenberg tumor.
  • The diverse problems in differential diagnosis of carcinoid tumor (provoked by nested, acinar, and other patterns, including folliclelike spaces) are then reviewed.
  • The section on breast cancer emphasizes that, although usually a manifestation of late stage disease and often not bulky in the ovaries, metastatic breast cancer may form large masses which can represent the clinical presentation.
  • The section on lung tumors largely reflects information in a recent paper that small cell carcinoma and adenocarcinoma are the lung cancers that spread to the ovary most commonly.
  • The extremely broad differential diagnosis posed by metastatic malignant melanoma ranging from that of an oxyphilic tumor, to a small cell tumor, to a follicle-forming neoplasm, is then considered.
  • The sections on renal cell carcinoma and other urinary tract neoplasms emphasize the differential diagnosis of metastatic clear cell carcinoma and primary clear cell carcinoma, an issue usually resolvable by an awareness of the various features of the ovarian variant, rarely or never seen in the renal variant.
  • The section on metastatic sarcomas discusses endometrial stromal sarcomas, gastrointestinal stromal neoplasms, and miscellaneous other sarcomas.
  • The endometrial stromal tumors are problematic largely because the history of a primary tumor may be remote, in the ovaries the typical growth and vascular pattern of endometrial stromal neoplasms is not always conspicuous, and some endometrial stromal sarcomas in the ovary show sex cordlike patterns of growth.
  • Recent information has indicated that gastrointestinal stromal tumors may rarely have significant ovarian manifestations and if the primary neoplasm is overlooked, the ovarian tumor may be misdiagnosed, usually as an ovarian fibromatous tumor, but potentially as another primary neoplasm.
  • The final neoplasms considered are malignant mesothelioma and the desmoplastic small round cell tumor.
  • The microscopic features of malignant mesothelioma are so different from those of primary ovarian carcinoma in most instances that the diagnosis should be readily established on routine microscopic evaluation.
  • The differential diagnosis of the desmoplastic small round cell tumor is more complex because of the greater overlap with the many other small cell malignant tumors that may involve the ovaries primarily or secondarily.
  • Nonetheless, differences exist in most cases and awareness of the entity should lead to consideration of the desmoplastic neoplasm, particularly in a young female.
  • However, as pointed out in brief concluding remarks, despite the aid of that modality, as in surgical pathology overall, careful consideration of the clinical background, distribution of disease, gross characteristics and spectrum of routine microscopic findings, will lead to the correct diagnosis in the majority of cases and at the very least lead to formulation of a considered differential diagnosis such that use of special techniques may be judicious and those results placed in context of the time-honored clinical and pathologic features.
  • [MeSH-major] Carcinoma / secondary. Krukenberg Tumor / secondary. Ovarian Neoplasms / secondary

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  • (PMID = 17452813.001).
  • [ISSN] 1072-4109
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Historical Article; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 67
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5. Ahmad J, Andrabi SI, Thekkinkattil DK, Rathore MA: Adenocarcinoid tumour of the appendix masquerading as acute appendicitis: a word of caution. Ulus Travma Acil Cerrahi Derg; 2008 Apr;14(2):167-9
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  • [Title] Adenocarcinoid tumour of the appendix masquerading as acute appendicitis: a word of caution.
  • Adenocarcinoids are rare and aggressive tumors with histological features of both carcinoid tumor and adenocarcinoma.
  • We report a case of a 32-year-old man with diffuse infiltration of the appendix wall and mesoappendix by an adenocarcinoid.
  • We suggest that patients with diffuse appendiceal involvement require an aggressive surgical therapy and follow-up.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / diagnosis

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  • (PMID = 18523911.001).
  • [ISSN] 1306-696X
  • [Journal-full-title] Ulusal travma ve acil cerrahi dergisi = Turkish journal of trauma & emergency surgery : TJTES
  • [ISO-abbreviation] Ulus Travma Acil Cerrahi Derg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Turkey
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6. Costantini M, Montalti R, Rossi G, Luisa L, Masetti M, Di Benedetto F, Giorgio G: Adenocarcinoid tumor of the extrahepatic biliary tract. Int J Surg Pathol; 2008 Oct;16(4):455-7
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  • [Title] Adenocarcinoid tumor of the extrahepatic biliary tract.
  • The term adenocarcinoid was first coined by Warkel et al in 1978 to describe a group of uncommon low-grade malignant appendiceal tumors with morphologic and histochemical evidence of both glandular (adenocarcinoma) and neuroendocrine (carcinoid) differentiation for which several terms have been used in the past.
  • Although the appendix is the most frequent site of this tumor, similar neoplasms have been reported also in other sites, such as colon, gallbladder, Vater's ampulla, and stomach.
  • Provided that it can metastasize, a recent meta-analysis on appendiceal adenocarcinoids showed that right hemicolectomy is not required when the tumor is completely excised and there is no cecal involvement.
  • In this article, the clinicopathologic features of an adenocarcinoid tumor occurring in the extrahepatic biliary tract with infiltration of the common hepatic duct wall that, to the best of our knowledge, represents the first report in this site is described.
  • [MeSH-major] Adenocarcinoma / pathology. Biliary Tract Neoplasms / pathology. Carcinoid Tumor / pathology
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Immunohistochemistry. Klatskin Tumor / pathology. Middle Aged. Ovarian Cysts / complications. Sjogren's Syndrome / complications. Tonsillectomy

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  • (PMID = 18480386.001).
  • [ISSN] 1066-8969
  • [Journal-full-title] International journal of surgical pathology
  • [ISO-abbreviation] Int. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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7. Chetty R, Serra S: Lipid-rich and clear cell neuroendocrine tumors ("carcinoids") of the appendix: potential confusion with goblet cell carcinoid. Am J Surg Pathol; 2010 Mar;34(3):401-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Lipid-rich and clear cell neuroendocrine tumors ("carcinoids") of the appendix: potential confusion with goblet cell carcinoid.
  • The so-called clear cell change has been described in neuroendocrine tumors at several locations.
  • Those associated with von Hippel Lindau disease are pathognomonically "clear" and the cytoplasmic appearance has been ascribed to intracytoplasmic lipid.
  • However, lipid has not been demonstrated in all cases of clear cell carcinoid tumors.
  • Such variants have not been described in carcinoid tumors of the appendix and cases with a prominent proportion of clear or more correctly, lipid-rich cytoplasm may bear a superficial resemblance to goblet cell carcinoid and/or signet ring adenocarcinoma.
  • Seven cases, in 5 females and 2 males ranging in age from 22 to 65 years, were noted to have a population of lipid-rich and vacuolated clear cells accounting for 25% or more of the tumor population.
  • The carcinoid tumors were incidental in all cases with 4 of patients presenting with appendicitis, 2 with concomitant mucinous cystadenocarcinomas of the appendix and 1 with an adenocarcinoma of the ascending colon.
  • Morphologically, the tumors had a nested and trabecular pattern and were composed of an admixture of microvesicular and clear lipid-rich cells.
  • None of the patients have shown evidence of recurrent disease.
  • The importance of recognizing this variant of carcinoid tumor in the appendix is to avoid confusion with goblet cell carcinoid tumors with or without a signet ring adenocarcinoma.
  • The presence of multi-vacuolated, foamy and clear cells, some resembling signet ring or goblet cells, in otherwise classic carcinoid tumors is rare but should be considered in this context in the appendix.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Lipids / analysis
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Cytoplasm / chemistry. Cytoplasm / pathology. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Incidental Findings. Male. Middle Aged. Prognosis. Vacuoles / chemistry. Vacuoles / pathology. Young Adult

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  • (PMID = 20139759.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Lipids
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8. Erbil Y, Barbaros U, Kapran Y, Yanik BT, Bozbora A, Ozarmağan S: Synchronous carcinoid tumour of the small intestine and appendix in the same patient. West Indian Med J; 2007 Mar;56(2):187-9
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  • [Title] Synchronous carcinoid tumour of the small intestine and appendix in the same patient.
  • Carcinoid tumours have been reported in a wide range of organs but most frequently involve the gastrointestinal tract.
  • Many of these carcinoid tumours are associated with metachronous and synchronous lesions of another histological type.
  • Primary carcinoid tumours of the different organ in the same patient is rare.
  • In this paper, the authors present a case with synchronous carcinoid tumour of the small intestine and appendix in the same patient.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / pathology. Cecal Neoplasms / diagnosis. Ileal Neoplasms / diagnosis. Intestine, Small / pathology

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  • (PMID = 17910153.001).
  • [ISSN] 0043-3144
  • [Journal-full-title] The West Indian medical journal
  • [ISO-abbreviation] West Indian Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Jamaica
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9. Dall'Igna P, Ferrari A, Luzzatto C, Bisogno G, Casanova M, Alaggio R, Terenziani M, Cecchetto G: Carcinoid tumor of the appendix in childhood: the experience of two Italian institutions. J Pediatr Gastroenterol Nutr; 2005 Feb;40(2):216-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Carcinoid tumor of the appendix in childhood: the experience of two Italian institutions.
  • OBJECTIVES: Although rare, carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in children and adolescents.
  • It is usually an incidental finding after a laparotomy for appendectomy, with a frequency of 2 to 5 cases per 1000 appendectomies.
  • The experience with 14 cases of carcinoid reported in the appendix is described.
  • METHODS AND RESULTS: In six patients the tumor measured 1 cm or less; only in one patient did it measure 2 cm.
  • In three patients the tumor measured between 1 and 2 cm and in four the size was not known.
  • All tumors were discovered by chance, and three patients underwent further surgery as a result of suspected involvement of the margins.
  • All the patients were alive with no evidence of disease at 24 to 214 months from diagnosis.
  • In our experience, both patients with local invasiveness and the patient with a tumor larger than 2 cm had good outcomes.
  • Ileocolectomy performed in the patient with a 2-cm tumor and in another two patients with smaller tumors did not demonstrate residual disease.
  • Although the need for right hemicolectomy still remains controversial for tumors measuring more than 2 cm, the approach may be nonaggressive in case of tumors invading the serosa and the periappendiceal fat.
  • Nonaggressive treatment has been suggested by some authors in cases of tumors larger than 2 cm; however, larger series need to be evaluated.
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / diagnosis
  • [MeSH-minor] Adolescent. Child. Colectomy / methods. Female. Humans. Ileus / surgery. Male. Neoplasm Invasiveness. Neoplasm Recurrence, Local / epidemiology. Prognosis. Treatment Outcome


10. Butte JM, García-Huidobro MA, Torres J, Duarte I, Zúñiga A, Llanos O: [Long-term survival in carcinoid tumour of the appendix. An analysis of 8903 appendectomies]. Gastroenterol Hepatol; 2009 Oct;32(8):537-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Long-term survival in carcinoid tumour of the appendix. An analysis of 8903 appendectomies].
  • [Transliterated title] Evaluación de la sobrevida a largo plazo del carcinoide del apéndice cecal. Un análisis a partir de 8.903 apendicectomías.
  • INTRODUCTION: Appendiceal carcinoids are the most frequent tumors of the appendix and are usually detected as an incidental finding in the final pathology report.
  • The aim of this study was to evaluate the clinical and pathological characteristics, surgical treatment and long-term survival in patients with an appendiceal carcinoid tumor.
  • MATERIAL AND METHODS: We performed a retrospective study of patients treated from 1980 to 2007 with a pathological diagnosis of appendiceal carcinoid tumor.
  • RESULTS: In this period, 8903 appendectomies were performed.
  • Carcinoid tumor was diagnosed in 40 patients (25 women and 15 men), aged 37+/-18 years old.
  • The tumor was localized in the distal third of the appendix in 24 patients (60%), and the size of the tumor was less than 1cm in 28 (70%).
  • Twenty-nine (72.5%) of these tumors infiltrated the subserosal and/or the serosal layer.
  • CONCLUSIONS: Appendiceal carcinoid tumor is usually detected incidentally in the pathological analysis in patients undergoing surgery for suspected appendicitis.
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / mortality. Appendiceal Neoplasms / surgery. Carcinoid Tumor / mortality. Carcinoid Tumor / surgery

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  • (PMID = 19616872.001).
  • [ISSN] 0210-5705
  • [Journal-full-title] Gastroenterología y hepatología
  • [ISO-abbreviation] Gastroenterol Hepatol
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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11. Hristov AC, Young RH, Vang R, Yemelyanova AV, Seidman JD, Ronnett BM: Ovarian metastases of appendiceal tumors with goblet cell carcinoidlike and signet ring cell patterns: a report of 30 cases. Am J Surg Pathol; 2007 Oct;31(10):1502-11
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  • [Title] Ovarian metastases of appendiceal tumors with goblet cell carcinoidlike and signet ring cell patterns: a report of 30 cases.
  • Synchronous and metachronous ovarian and appendiceal mucinous tumors are often the subject of diagnostic consultations in gynecologic pathology practices to address whether the tumors are independent neoplasms or related as primary and metastasis.
  • The ovarian tumors were bilateral in 25 of 28 cases with data on both ovaries and were typically large (mean/median: 14 cm, range: 4.5 to 24.0 cm).
  • The appendices were often firm or thickened but usually did not have a discrete measurable tumor and were not notably enlarged; microscopically, transmural invasion was present in all of them.
  • The ovarian and appendiceal tumors exhibited a variety of patterns of differentiation, including signet ring cell and glandular, with all displaying some goblet cell carcinoidlike patterns (nests, islands, cords, or cryptlike tubules with goblet cells); teratomatous elements were not identified in any ovarian tumors.
  • Chromogranin was expressed in 7 of 19 ovarian tumors (mean/median: 6.3%/0%; range: 0% to 20%) and synaptophysin was expressed in 4 of 18 of these (mean/median: 7.8%/0%; range: 0% to 90%).
  • Chromogranin was expressed in 6 of 16 appendiceal tumors (mean/median: 11.9%/0%; range: 0% to 70%) and synaptophysin was expressed in 6 of 15 of these (mean/median: 16.7%/0%; range: 0% to 90%).
  • Follow-up was available for 25 patients: 17 died of disease at intervals ranging from 4 to 47 months (mean/median: 18/16) and 8 were alive with disease at 1 to 25 months (mean/median: 11/10); median survival was 19 months and the 1-year and 2-year survival rates were 63% and 34%, respectively.
  • The clinicopathologic features of these ovarian tumors indicate they should be labeled as metastatic appendiceal adenocarcinomas rather than as goblet cell carcinoid tumors both to reflect their behavior and help distinguish them from the rare true primary ovarian goblet cell carcinoid tumors.
  • As the ovarian tumors have appreciable components of signet ring cells they qualify as Krukenberg tumors.
  • In cases in which the primary tumor is not already evident, their "goblet cell carcinoidlike" patterns should direct attention to the appendix as a possible source.
  • [MeSH-major] Adenocarcinoma / secondary. Appendiceal Neoplasms / pathology. Carcinoid Tumor / secondary. Carcinoma, Signet Ring Cell / secondary. Ovarian Neoplasms / secondary
  • [MeSH-minor] Adult. Aged. Biomarkers, Tumor / metabolism. Chromogranins / metabolism. Female. Humans. Middle Aged. Survival Rate. Synaptophysin / metabolism

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  • (PMID = 17895750.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromogranins; 0 / Synaptophysin
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12. Liu E, Telem DA, Hwang J, Warner RR, Dikman A, Divino CM: The clinical utility of Ki-67 in assessing tumor biology and aggressiveness in patients with appendiceal carcinoids. J Surg Oncol; 2010 Sep 15;102(4):338-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The clinical utility of Ki-67 in assessing tumor biology and aggressiveness in patients with appendiceal carcinoids.
  • BACKGROUND/OBJECTIVE: To elucidate the correlation of Ki-67 with tumor biology and survival in appendiceal carcinoid tumors.
  • METHOD: A retrospective chart review conducted on 51 patients with appendiceal carcinoid tumors who underwent surgical intervention from 1991 to 2008.
  • MIB-1 index was categorized according to the World Health Organization (WHO) classification.
  • RESULT: Of the 51 patients, 32 had tumors <2 cm; 3 >2 cm; and 16 with unspecified tumor size.
  • Increased MIB proliferative index did not significantly correlate with increasing tumor size (P = 0.426).
  • Twelve patients had metastatic disease on presentation: 9 had MIB-1 index <2%, 1 had index 2-15% and 2 with index >15%.
  • No correlation was demonstrated by MIB index and tumor size or presentation with metastatic disease.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Ki-67 Antigen / analysis
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Invasiveness

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  • [Copyright] (c) 2010 Wiley-Liss, Inc.
  • (PMID = 20607756.001).
  • [ISSN] 1096-9098
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Ki-67 Antigen
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13. Albores-Saavedra J, Henson DE, Batich K: Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix. Am J Surg Pathol; 2009 Aug;33(8):1259-60; author reply 1260-1
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.
  • [MeSH-major] Adenocarcinoma / classification. Adenocarcinoma / pathology. Appendiceal Neoplasms / classification. Appendiceal Neoplasms / pathology. Carcinoid Tumor / classification. Carcinoid Tumor / pathology
  • [MeSH-minor] Humans. Incidental Findings. Male. Middle Aged

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  • [CommentOn] Am J Surg Pathol. 2008 Oct;32(10):1429-43 [18685490.001]
  • (PMID = 19471156.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Comment; Letter
  • [Publication-country] United States
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14. Azordegan N, Yazdankhah A, Moghadasian MH: A rare case of coexistence of carcinoid tumor of appendix vermicularis and ileal endometriosis. Arch Gynecol Obstet; 2009 Feb;279(2):183-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A rare case of coexistence of carcinoid tumor of appendix vermicularis and ileal endometriosis.
  • BACKGROUND: Carcinoid tumor is the most common tumor of appendix with overall good prognosis.
  • CASE REPORT: We here report the coexistence of carcinoid tumor of appendix and ileal endometriosis in a 37-year-old nulliparous woman who came to the emergency room with right lower abdominal pain mimicking acute appendicitis.
  • The main clinical and laboratory findings included fever, leukocytosis and elevated ESR.
  • With preoperative suspicion of acute appendicitis, laparatomy was performed and revealed apparently normal looking appendix, along with a nodule in the terminal ileum.
  • Both the appendix and ileal nodule were removed.
  • Histological examinations revealed carcinoid tumor of appendix and ileal endometriosis.
  • CONCLUSION: Many cases of carcinoid tumor of the appendix and ileal endometriosis are diagnosed incidentally.
  • [MeSH-major] Appendiceal Neoplasms / complications. Carcinoid Tumor / complications. Endometriosis / complications. Ileal Diseases / complications


15. Northrop JA, Lee JH: Large bowel carcinoid tumors. Curr Opin Gastroenterol; 2007 Jan;23(1):74-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Large bowel carcinoid tumors.
  • PURPOSE OF REVIEW: Gastrointestinal carcinoids comprise 90% of all carcinoid tumors and all carcinoids have malignant potential.
  • This review focuses on the morphology, prognosis, detection and treatment for appendiceal, colonic and rectal carcinoids.
  • RECENT FINDINGS: Computed tomography exists as an initial examination for the primary carcinoid tumor as well as for metastases.
  • Octreotide scanning has a sensitivity of primary tumor detection of 90%; additionally, I- or I-meta-iodobenzylguanidine scanning and tracer-specific positron emission tomographic scans have demonstrated encouraging results.
  • Further advancements in treatment with tumor-targeted therapy and biochemical evaluation of carcinoids have shown promise.
  • SUMMARY: The recent progress with scintigraphic and radiologic modalities has provided better means for diagnosis of primary and metastatic carcinoid tumors.
  • These newly discovered diagnostic modalities have been more encouraging than the recent treatment approaches that have been studied with regard to metastatic carcinoids.
  • While surgery remains the mainstay of treatment of nonmetastatic carcinoid, there have been studies for various medical treatments of metastatic disease.
  • Unfortunately, there have been disappointing results with regard to improvement of tumor response and patient survival, but a foundation has been established for future trials employing alternative agents and exploration of combination therapies.
  • [MeSH-major] Carcinoid Tumor / diagnosis. Carcinoid Tumor / therapy. Intestinal Neoplasms / diagnosis. Intestinal Neoplasms / therapy. Intestine, Large / pathology

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  • (PMID = 17133089.001).
  • [ISSN] 0267-1379
  • [Journal-full-title] Current opinion in gastroenterology
  • [ISO-abbreviation] Curr. Opin. Gastroenterol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 25
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16. Leanza S, Bekheit M, Coco D, Bellia A, Ferrara F, Sarvà S, Pappalardo A, Piazza L: Carcinoma of the appendix and its natural history in relation to surgical management. A case report. Chir Ital; 2009 Sep-Dec;61(5-6):597-600
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Carcinoma of the appendix and its natural history in relation to surgical management. A case report.
  • Epithelial tumours of the appendix have been classified into four distinct types: carcinoids, mucinous adenocarcinoma (often called mucinous cystadenocarcinoma or malignant mucocele), colonic-type adenocarcinoma, and adenocarcinoids with a dual cell origin.
  • We report a case of a woman who presented to our emergency unit with a history of acute appendicitis and who had undergone an appendectomy.
  • The histopathological analysis showed the presence of a well differentiated mucinous adenocarcinoma, infiltrating the full thickness of the appendix wall.
  • [MeSH-major] Adenocarcinoma, Mucinous / diagnosis. Adenocarcinoma, Mucinous / surgery. Appendiceal Neoplasms / diagnosis. Appendiceal Neoplasms / surgery

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  • (PMID = 20380264.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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17. Patel S, Heetun M, Gurjar SV, Szakacs S: A rare case of intra-cardiac metastasis from an appendiceal carcinoid tumour without liver metastases. Int J Colorectal Dis; 2009 Aug;24(8):993-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A rare case of intra-cardiac metastasis from an appendiceal carcinoid tumour without liver metastases.
  • [MeSH-major] Appendiceal Neoplasms / complications. Carcinoid Heart Disease / etiology. Carcinoid Tumor / complications. Myocardium / pathology
  • [MeSH-minor] Appendectomy. Autopsy. Cardiopulmonary Resuscitation. Fatal Outcome. Female. Heart Arrest / etiology. Humans. Liver Neoplasms / secondary. Middle Aged. Palliative Care. Treatment Outcome

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  • (PMID = 19387658.001).
  • [ISSN] 1432-1262
  • [Journal-full-title] International journal of colorectal disease
  • [ISO-abbreviation] Int J Colorectal Dis
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Germany
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18. Taal BG, Smits M: Developments in diagnosis and treatment of metastatic midgut carcinoid tumors. A review. Minerva Gastroenterol Dietol; 2005 Dec;51(4):335-44
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Developments in diagnosis and treatment of metastatic midgut carcinoid tumors. A review.
  • Carcinoids are neuroendocrine tumours derived from enterochromaffin cells which are widely distributed in the body and may, therefore, arise from any site.
  • Localisation in the gastrointestinal tract is the most frequent, among which the appendiceal involvement is often found at laparoscopy for appendicitis and the small bowel is known for the liver metastases with the production of serotonin causing the characteristic carcinoid syndrome with diarrhoea and flushes.
  • The overall incidence of carcinoid disease has increased in the past decades, but whether this is a true increase or due to early detection or better recognition at pathology is not known.
  • The prognosis of metastatic carcinoid tumours has improved during the last decade resulting in a 5 year survival of approximately 50% in the Netherlands.
  • Due to a longer survival, complications such as carcinoid heart disease and new metastatic patterns like skin and bone metastases may become a more important feature in carcinoid disease.
  • New developments are in the field of diagnostics (fine-tuning of the pathology, videocapsule endoscopy to find the primary tumour, positron emission tomography [PET] scanning) and treatment options (radiofrequency ablation, radioactive octreotide, meta-iodobenzylguanidine combinations).
  • The new serum marker of carcinoid, chromogranin A, may play an important role in the follow-up and NT-proBNP for the detection of heart problems.
  • Combining new diagnostic and treatment modalities in metastatic carcinoid patients may result in a better quality of life and a longer survival.
  • [MeSH-major] Carcinoid Tumor / diagnosis. Carcinoid Tumor / therapy. Intestinal Neoplasms / diagnosis. Intestinal Neoplasms / therapy

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  • (PMID = 16282961.001).
  • [ISSN] 1121-421X
  • [Journal-full-title] Minerva gastroenterologica e dietologica
  • [ISO-abbreviation] Minerva Gastroenterol Dietol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 57
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19. Krysiak R, Okopień B, Herman ZS: [Current concepts on diagnosis and treatment of carcinoid]. Przegl Lek; 2007;64(2):103-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Current concepts on diagnosis and treatment of carcinoid].
  • Carcinoid tumours are relatively rare neuroendocrine neoplasms that often present as diagnostic dilemmas due to obscure or non-specific symptomatology.
  • Carcinoids occur most frequently in the gastrointestinal system, where they are most common in the small intestine, appendix, and rectum, and in the bronchi.
  • Although, the majority are nonfunctional, some carcinoids can cause so called classical or atypical carcinoid syndrome and sometimes also paraneoplastic syndromes.
  • Carcinoid tumours often present with metastatic disease.
  • Recent years have brought new developments in the field of their diagnostic and treatment options.
  • Despite the fact that many advances have been made in both the basic science and clinical areas, the optimal treatment of carcinoid tumours is still a matter of debate.
  • In this article, the pathogenesis, clinical aspects, classification, diagnosis and treatment of carcinoids are reviewed including the latest advances in each area.
  • [MeSH-major] Carcinoid Tumor / diagnosis. Carcinoid Tumor / therapy. Gastrointestinal Neoplasms / diagnosis. Gastrointestinal Neoplasms / therapy
  • [MeSH-minor] Appendiceal Neoplasms / diagnosis. Appendiceal Neoplasms / therapy. Bronchial Neoplasms / diagnosis. Bronchial Neoplasms / therapy. Humans. Rectal Neoplasms / diagnosis. Rectal Neoplasms / therapy

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  • (PMID = 17892042.001).
  • [ISSN] 0033-2240
  • [Journal-full-title] Przegla̧d lekarski
  • [ISO-abbreviation] Prz. Lek.
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Poland
  • [Number-of-references] 46
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20. Chetty R, Klimstra DS, Henson DE, Albores-Saavedra J: Combined classical carcinoid and goblet cell carcinoid tumor: a new morphologic variant of carcinoid tumor of the appendix. Am J Surg Pathol; 2010 Aug;34(8):1163-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Combined classical carcinoid and goblet cell carcinoid tumor: a new morphologic variant of carcinoid tumor of the appendix.
  • Carcinoid tumors are the most common neoplasms of the appendix.
  • Goblet cell carcinoid has been regarded as a distinctive tumor type, not related to classic carcinoids, and to our knowledge combinations of these 2 tumor types have not been described in detail.
  • In this report, we describe 5 cases of combined classical carcinoid and goblet cell carcinoid (GCC) tumors of the appendix.
  • Four men and 1 woman, (mean age 53.4 y) presented with acute appendicitis (4 cases), whereas 1 presented with a pelvic mass owing to widespread pelvic disseminated disease.
  • The tumors (0.6 to 6.0 cm) were located in the mid-portion and the tip of the appendix.
  • Four patients were treated with right hemicolectomies (the patient with disseminated pelvic and ovarian metastases also had a pelvic exenteration), and 1 was treated with an appendectomy only.
  • Four patients are alive and asymptomatic, whereas the patient with disseminated pelvic disease died 6 months after surgery.
  • All 5 appendiceal tumors had microscopic features of both classical carcinoid and GCC, either intimately admixed or separate but closely apposed.
  • The extent of the 2 components varied, with classical carcinoid representing 60% to 90% of the tumor.
  • The pelvic soft tissue and ovarian metastases in case 4 consisted predominantly of a signet ring cell carcinoma with a minor component of goblet cells and was interpreted as an adenocarcinoma ex-GCC.
  • In view of the fact that these combined carcinoid tumors appear to behave more as goblet cell carcinoids, detailed microscopic examination of classical carcinoid tumors of the appendix is suggested and larger series with longer follow-up is required to ascertain the true biologic potential of this unique form of combined carcinoid tumor of the appendix.
  • The occurrence of both carcinoid types in the same appendices suggests a closer histogenetic relationship than previously believed, although the possibility that the 2 components represent separate, independent primaries ("collision tumors") can also be considered.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Goblet Cells / pathology. Mixed Tumor, Malignant / pathology
  • [MeSH-minor] Appendectomy. Biomarkers, Tumor / analysis. Colectomy. Female. Humans. Immunohistochemistry. Male. Middle Aged. Mitotic Index. Neoplasm Invasiveness. Treatment Outcome

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  • [CommentIn] Am J Surg Pathol. 2011 Aug;35(8):1248-50 [21753706.001]
  • (PMID = 20631606.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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21. Bucher P, Gervaz P, Ris F, Oulhaci W, Inan I, Morel P: Laparoscopic versus open resection for appendix carcinoid. Surg Endosc; 2006 Jun;20(6):967-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic versus open resection for appendix carcinoid.
  • BACKGROUND: Since an increasing number of appendectomies are performed via laparoscopy, it is crucial to determine the impact of this approach on appendix carcinoid (AC) outcome.
  • The goal of this study was to compare results of laparoscopic (LAP) versus open (OP) appendectomy for AC according to intend to treat approach.
  • RESULTS: Most cases had associated acute appendicitis (64%).
  • Median carcinoid size was 1.1 cm (range, 0.3-5) and 0.4 cm (range, 0.2-3) in the LAP and OP groups, respectively.
  • Two patients died in the OP group, one due to metastatic carcinoid and the other due to metachronous colorectal cancer.
  • CONCLUSION: Laparoscopic appendectomy is a safe procedure for AC, with carcinologic and long-term results similar to those of conventional appendectomy.
  • Prognosis of AC appears more dependent on carcinoid malignant potential or associated tumors.
  • [MeSH-major] Appendectomy / methods. Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery. Laparoscopy
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Follow-Up Studies. Humans. Incidence. Male. Middle Aged. Neoplasms, Multiple Primary / epidemiology. Neoplasms, Second Primary / epidemiology. Retrospective Studies

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  • (PMID = 16738993.001).
  • [ISSN] 1432-2218
  • [Journal-full-title] Surgical endoscopy
  • [ISO-abbreviation] Surg Endosc
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Germany
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22. Koopmans NG, Kwee HW, Heemskerk J: [Acute appendicitis with goblet cell carcinoid]. Ned Tijdschr Geneeskd; 2010;154:A1154
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Acute appendicitis with goblet cell carcinoid].
  • The patient underwent an appendicectomy of the inflamed appendix with periappendicular infiltrate.
  • However, histological examination revealed a small goblet cell carcinoid, histological group C, with acute inflammation.
  • Several tumour cells were present in a lymph duct at the appendix base.
  • The treatment should be based also on the histological classification.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Appendicitis / diagnosis. Carcinoid Tumor / diagnosis

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  • (PMID = 20619032.001).
  • [ISSN] 1876-8784
  • [Journal-full-title] Nederlands tijdschrift voor geneeskunde
  • [ISO-abbreviation] Ned Tijdschr Geneeskd
  • [Language] dut
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Netherlands
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23. Srivastava A, Hornick JL: Immunohistochemical staining for CDX-2, PDX-1, NESP-55, and TTF-1 can help distinguish gastrointestinal carcinoid tumors from pancreatic endocrine and pulmonary carcinoid tumors. Am J Surg Pathol; 2009 Apr;33(4):626-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Immunohistochemical staining for CDX-2, PDX-1, NESP-55, and TTF-1 can help distinguish gastrointestinal carcinoid tumors from pancreatic endocrine and pulmonary carcinoid tumors.
  • Well-differentiated neuroendocrine tumors (WDNET) of the gastrointestinal tract, pancreas, and lung are histologically similar.
  • Thus, predicting the site of origin of a metastasis is not possible on morphologic grounds.
  • Prior immunohistochemical studies of WDNET have yielded conflicting results, and pancreatic and duodenal homeobox factor-1 (PDX-1) has not previously been evaluated in this context.
  • In total, 64 gastrointestinal carcinoids (5 stomach; 5 duodenum; 31 ileum; 11 appendix; and 12 rectum); 39 pancreatic endocrine tumors (PET); and 20 pulmonary carcinoid tumors were studied.
  • Ileal carcinoids were exclusively positive for CDX-2 (30/31) and negative for all other markers.
  • Appendiceal carcinoids were uniformly positive for CDX-2 (11/11).
  • All rectal carcinoids were negative for CDX-2 and TTF-1; 2/12 were positive for PDX-1, and 1/12 for NESP-55.
  • The gastric and duodenal carcinoids were only positive for PDX-1 (7/10).
  • TTF-1 positivity was confined to pulmonary carcinoids (7/20); 1/20 was positive for NESP-55; and all were negative for CDX-2 and PDX-1.
  • TTF-1 positivity was confined to pulmonary carcinoids in our study but was present in only about a third of cases.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Carcinoid Tumor / diagnosis. Gastrointestinal Neoplasms / diagnosis. Lung Neoplasms / diagnosis. Neoplasm Proteins / metabolism. Pancreatic Neoplasms / diagnosis

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  • (PMID = 19065104.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / Homeodomain Proteins; 0 / Neoplasm Proteins; 0 / TTF1 protein, human; 0 / Trans-Activators; 0 / pancreatic and duodenal homeobox 1 protein; 156560-97-3 / Cdx-2-3 protein; EC 3.6.1.- / GNAS protein, human; EC 3.6.5.1 / GTP-Binding Protein alpha Subunits, Gs
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24. Shapiro R, Eldar S, Sadot E, Venturero M, Papa MZ, Zippel DB: The significance of occult carcinoids in the era of laparoscopic appendectomies. Surg Endosc; 2010 Sep;24(9):2197-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The significance of occult carcinoids in the era of laparoscopic appendectomies.
  • BACKGROUND: We present data acquired in our institution about the incidence of incidental appendiceal carcinoids over a period of 16 years.
  • The possibility of occult carcinoids raises the question of appendectomy of a noninflamed appendix during diagnostic laparoscopy for suspected appendicitis.
  • METHODS: We performed a retrospective chart analysis of the surgical registry of a university-affiliated tertiary care center of a major population area for the past 16 years.
  • Outcome measures were the incidence of incidental carcinoids of the appendix found during appendectomies and whether the introduction of laparoscopic appendectomy should alter the surgical management of a normal-appearing appendix.
  • RESULTS: A total of 20 carcinoid appendices were resected by open surgery and 17 by laparoscopy.
  • The diagnosis of a carcinoid tumor was not suspected in any patient before the operation, nor was a tumor identified at the time of the operation.
  • In 6 (16%) patients the appendix appeared normal at the time of the operation.
  • CONCLUSIONS: It has long been the standard of care to remove any appendix found in laparotomy for suspected appendicitis, but it is not clear what should be done during laparoscopy for suspected appendicitis when the appendix appears normal.
  • Our data confirm the presence of occult carcinoids in normal-appearing appendices.
  • Further studies are needed to determine the clinical significance of this finding.
  • [MeSH-major] Appendectomy / methods. Appendiceal Neoplasms / epidemiology. Appendiceal Neoplasms / surgery. Carcinoid Tumor / epidemiology. Carcinoid Tumor / surgery. Laparoscopy / methods
  • [MeSH-minor] Adolescent. Adult. Child. Female. Humans. Incidence. Incidental Findings. Male. Middle Aged. Registries. Retrospective Studies

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  • (PMID = 20174936.001).
  • [ISSN] 1432-2218
  • [Journal-full-title] Surgical endoscopy
  • [ISO-abbreviation] Surg Endosc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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25. Bamboat ZM, Berger DL: Is right hemicolectomy for 2.0-cm appendiceal carcinoids justified? Arch Surg; 2006 Apr;141(4):349-52; discussion 352
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  • [Title] Is right hemicolectomy for 2.0-cm appendiceal carcinoids justified?
  • HYPOTHESIS: We believe right hemicolectomy (RHC) is not necessary in patients with an appendiceal carcinoid greater than 2.0 cm.
  • DESIGN: A retrospective review of patients with a histologically confirmed appendiceal carcinoid from April 1, 1980, to February 28, 2005, and an analysis of the literature.
  • PATIENTS: Forty-eight patients (34 females and 14 males) with a histologically confirmed diagnosis of appendiceal carcinoid were included in the study.
  • Appendiceal carcinoid was diagnosed incidentally in all 48 patients.
  • Postoperative follow-up and disease-free survival were confirmed in 33 patients via medical record review.
  • MAIN OUTCOME MEASURES: We assessed the relationship between survival, tumor size, and the role of RHC vs appendectomy alone.
  • RESULTS: Four patients in our series underwent secondary RHC and lymph node dissection for tumors greater than 2.0 cm, and none had positive lymph nodes.
  • Following review of the literature, we were unable to find any recent evidence of distant metastasis from carcinoids in patients already treated by appendectomy.
  • There seem to be no conclusive data to support the notion that RHC confers a survival benefit over appendectomy for carcinoids greater than 2.0 cm.
  • CONCLUSION: Appendiceal carcinoids greater than 2.0 cm can be managed effectively with simple appendectomy, given their low malignant potential and slow growth, obviating the need for RHC in this group of patients without affecting overall survival.
  • [MeSH-major] Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery. Colectomy / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Appendectomy. Child. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Postoperative Complications. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 16618891.001).
  • [ISSN] 0004-0010
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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26. Owen A, Moore O, Marven S, Roberts J: Interval laparoscopic appendectomy in children. J Laparoendosc Adv Surg Tech A; 2006 Jun;16(3):308-11
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  • Formation of an appendiceal mass or abscess may mitigate against urgent appendectomy during the acute stage, and conservative treatment followed by interval appendectomy has been advocated.
  • RESULTS: Thirty-six children, median age 8 years (range, 1-15 years) diagnosed with appendiceal mass or abscess were offered interval laparoscopic appendectomy by two surgeons in our institution: one patient declined interval laparoscopic appendectomy.
  • CONCLUSION: Interval laparoscopic appendectomy can be safely performed in children, is associated with a short hospital stay and minimal morbidity, analgesia, and scarring following conservative management of appendiceal mass or abscess.
  • Interval laparoscopic appendectomy eliminates the risk of recurrent appendicitis and serves to excise undiagnosed carcinoid tumors.

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  • (PMID = 16796448.001).
  • [ISSN] 1092-6429
  • [Journal-full-title] Journal of laparoendoscopic & advanced surgical techniques. Part A
  • [ISO-abbreviation] J Laparoendosc Adv Surg Tech A
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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27. West NE, Wise PE, Herline AJ, Muldoon RL, Chopp WV, Schwartz DA: Carcinoid tumors are 15 times more common in patients with Crohn's disease. Inflamm Bowel Dis; 2007 Sep;13(9):1129-34
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  • [Title] Carcinoid tumors are 15 times more common in patients with Crohn's disease.
  • BACKGROUND: The coexistence of intestinal neoplasms with Crohn's disease (CD) has been reported, but the evidence of an increased risk of carcinoid tumor with Crohn's disease has been mixed.
  • We present 4 patients with CD with associated carcinoid tumor.
  • The number of incidental carcinoid tumors in patients who underwent an appendectomy was used as a control.
  • RESULTS: Four cases of carcinoid tumor discovered in patients at resection for CD were identified.
  • None had metastatic disease or carcinoid syndrome.
  • These included 1 cecal (1 mm), 2 appendiceal (3 and 7 mm), and 1 transverse colon (7 mm) carcinoid tumors.
  • None of the carcinoid tumors were identified in regions of active Crohn's disease.
  • The incidence of carcinoid tumor in patients with Crohn's disease was 4 of 111 (3.6%).
  • In comparison, 3 of 1199 patients (0.25%) who had appendectomies were identified as having appendiceal carcinoid tumor.
  • Crohn's disease was associated with an increased incidence of carcinoid tumor; OR 14.9 (95% CI 2.5-102.5), P<0.0001.
  • CONCLUSIONS: There was a significantly increased incidence of carcinoid tumor in our Crohn's patients compared to the control patients.
  • None of the carcinoid tumors developed in areas of Crohn's disease.
  • This suggests that the development of carcinoid tumors may be secondary to distant proinflammatory mediators, rather than a local inflammatory effect from adjacent Crohn's disease.
  • Patients with CD may be at increased risk of developing a carcinoid tumor.
  • [MeSH-major] Carcinoid Tumor / diagnosis. Crohn Disease / diagnosis
  • [MeSH-minor] Adult. Appendectomy. Cohort Studies. Female. Humans. Inflammation. Inflammatory Bowel Diseases / diagnosis. Intestinal Neoplasms / complications. Intestinal Neoplasms / diagnosis. Intestinal Neoplasms / epidemiology. Male. Middle Aged. Odds Ratio. Time Factors

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  • [CommentIn] Inflamm Bowel Dis. 2008 Nov;14(11):1613-4 [18521905.001]
  • (PMID = 17538985.001).
  • [ISSN] 1078-0998
  • [Journal-full-title] Inflammatory bowel diseases
  • [ISO-abbreviation] Inflamm. Bowel Dis.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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28. Pham TH, Wolff B, Abraham SC, Drelichman E: Surgical and chemotherapy treatment outcomes of goblet cell carcinoid: a tertiary cancer center experience. Ann Surg Oncol; 2006 Mar;13(3):370-6
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  • [Title] Surgical and chemotherapy treatment outcomes of goblet cell carcinoid: a tertiary cancer center experience.
  • BACKGROUND: Goblet cell carcinoid (GCC) is a rare malignant tumor with distinct histological and clinical features.
  • RESULTS: The age at diagnosis (mean +/- SE) was 55 +/- 13 years.
  • The disease-specific 5-year survivals for stages I, II, III, and IV were 100%, 76%, 22%, and 14%, respectively; the overall mean survival was 47 +/- 3 months.
  • CONCLUSIONS: The prognosis for patients with GCC tumors correlates well with the American Joint Committee on Cancer stage at initial presentation.
  • Appendectomy alone seems adequate for stage I disease.
  • For staging purposes, right hemicolectomy is appropriate for T4 tumors or stage II to III disease provided that it can be performed with minimal risk.
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / drug therapy. Appendiceal Neoplasms / surgery. Carcinoid Tumor / drug therapy. Carcinoid Tumor / surgery
  • [MeSH-minor] Adult. Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Fluorouracil / administration & dosage. Humans. Leucovorin / administration & dosage. Male. Middle Aged. Neoplasm Staging. Prognosis. Prospective Studies. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 16485156.001).
  • [ISSN] 1068-9265
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] Q573I9DVLP / Leucovorin; U3P01618RT / Fluorouracil
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29. Pitiakoudis M, Kirmanidis M, Tsaroucha A, Christianakis E, Filippou D, Sivridis E, Simopoulos C: Carcinoid tumor of the appendix during pregnancy. A rare case and a review of the literature. J BUON; 2008 Apr-Jun;13(2):271-5
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  • [Title] Carcinoid tumor of the appendix during pregnancy. A rare case and a review of the literature.
  • We present a rare case of a carcinoid tumor of the appendix that was diagnosed during pregnancy in a 24-year-old female.
  • Open appendectomy was carried out and at the histological examination carcinoid was found in the surgical specimen.
  • Only few similar cases were found in the literature reporting appendiceal carcinoid tumor during pregnancy.
  • [MeSH-major] Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery. Pregnancy Complications, Neoplastic / pathology. Pregnancy Complications, Neoplastic / surgery


30. Bertuzzo VR, Coccolini F, Pinna AD: Peritoneal seeding from appendiceal carcinoma: A case report and review of the literature. World J Gastrointest Surg; 2010 Aug 27;2(8):265-9
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  • [Title] Peritoneal seeding from appendiceal carcinoma: A case report and review of the literature.
  • Non-carcinoid appendiceal malignancies are rare entities, representing less than 0.5% of all gastrointestinal malignancies.
  • Because of their rarity and particular biological behavior, a substantial number of patients affected by these neoplasms do not receive appropriate surgical resection.
  • In this report, we describe a rare case of primary signet-ring cell carcinoma of the appendix with peritoneal seeding which occurred in a 40-year old man admitted at the Emergency Surgery Department with the clinical suspicion of acute appendicitis.
  • This report offers a brief review of the literature and suggests an algorithm for the management of non-carcinoid appendiceal tumors with peritoneal dissemination.

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  • (PMID = 21160886.001).
  • [ISSN] 1948-9366
  • [Journal-full-title] World journal of gastrointestinal surgery
  • [ISO-abbreviation] World J Gastrointest Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC2999252
  • [Keywords] NOTNLM ; Appendiceal neoplasms / Hyperthermic intraperitoneal chemotherapy / Peritoneal carcinomatosis / Signet-ring cell carcinoma / Therapeutic algorithm
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31. Deschamps L, Couvelard A: Endocrine tumors of the appendix: a pathologic review. Arch Pathol Lab Med; 2010 Jun;134(6):871-5
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  • [Title] Endocrine tumors of the appendix: a pathologic review.
  • CONTEXT: Although rare, appendiceal endocrine tumors are the most common neoplasms of the appendix.
  • OBJECTIVE: To provide recent data that focus on the pathology of endocrine tumors of the appendix including classifications and guidelines for patient management.
  • DATA SOURCES: A review of the recent literature including TNM classifications and patient management guidelines.
  • CONCLUSIONS: Appendiceal endocrine tumors are separated into 2 main groups: classic endocrine tumors and goblet cell carcinoids.
  • Evaluation of their prognoses and risks of malignancy, according to these classifications, depends on several parameters including tumor size, proliferation rate, and infiltration of appendiceal wall and mesoappendix.
  • Most patients with classic endocrine tumors of the appendix have a favorable prognosis.
  • Indications for postappendectomy, complementary surgery, which are still controversial, especially for tumors between 1 and 2 cm, are presented and discussed.
  • In contrast, in patients presenting with a goblet cell carcinoid, a right hemicolectomy after the initial appendectomy is considered the standard surgical intervention.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoma, Neuroendocrine / pathology
  • [MeSH-minor] Appendectomy. Carcinoid Tumor / classification. Carcinoid Tumor / diagnosis. Carcinoid Tumor / pathology. Colectomy. Humans. Prognosis. World Health Organization

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  • (PMID = 20524865.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 41
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32. Yamada A, Yamamoto H, Arai O, Kiyosuke Y, Tsuji Y, Nakatsuji M, Fukatsu H, Miyoshi M, Kono H, Mouri H, Matsueda K, Tezen T, Shimamura J, Tsukayam C: [A case of goblet cell carcinoid of the appendix presented as a metastatic ovarian tumor and peritoneal dissemination, and diagnosed by autopsy]. Nihon Shokakibyo Gakkai Zasshi; 2005 Jul;102(7):905-11
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  • [Title] [A case of goblet cell carcinoid of the appendix presented as a metastatic ovarian tumor and peritoneal dissemination, and diagnosed by autopsy].
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / secondary. Ovarian Neoplasms / secondary. Peritoneal Neoplasms / secondary

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  • (PMID = 16038438.001).
  • [ISSN] 0446-6586
  • [Journal-full-title] Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
  • [ISO-abbreviation] Nihon Shokakibyo Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 12
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33. Debnath D, Rees J, Myint F: Are we missing diagnostic opportunities in cases of carcinoid tumours of the appendix? Surgeon; 2008 Oct;6(5):266-72
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  • [Title] Are we missing diagnostic opportunities in cases of carcinoid tumours of the appendix?
  • OBJECTIVE: Carcinoid tumour of appendix is an uncommon condition that can potentially give rise to a variation in management.
  • We aimed to assess the occurrence and mode of presentation of carcinoid tumour of appendix, and any variation of its management.
  • RESULTS: A total of 1941 appendicectomies took place.
  • Sixteen (0.82%) patients had carcinoid tumours.
  • The mean age of patients with carcinoid tumour (41.8 years) was significantly higher than those with non-carcinoid pathology (27.7 years) (p = 0.001).
  • The incidence of female patients was higher than the male amongst the carcinoid tumour group (female/male ratio 2.2).
  • None of the carcinoid tumours were identified at operation.
  • Eight patients as well as their general practitioners (53.3%) were unaware of the diagnosis.
  • CONCLUSIONS: Carcinoid tumour of the appendix remains an incidental diagnosis.
  • Patients with carcinoids were significantly older than non-carcinoid conditions.
  • There was a trend of increased occurrence of carcinoids amongst the females.
  • There was a wide variation in involving the multidisciplinary team, conveying the diagnosis to patients as well as their general practitioners, and follow-ups.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Appendiceal Neoplasms / surgery. Carcinoid Tumor / pathology. Carcinoid Tumor / surgery
  • [MeSH-minor] Adult. Appendectomy. Chi-Square Distribution. Female. Humans. Incidence. Incidental Findings. Male. Reoperation. Sex Factors

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  • (PMID = 18939372.001).
  • [ISSN] 1479-666X
  • [Journal-full-title] The surgeon : journal of the Royal Colleges of Surgeons of Edinburgh and Ireland
  • [ISO-abbreviation] Surgeon
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
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34. Khan MN, Moran BJ: Four percent of patients undergoing colorectal cancer surgery may have synchronous appendiceal neoplasia. Dis Colon Rectum; 2007 Nov;50(11):1856-9
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  • [Title] Four percent of patients undergoing colorectal cancer surgery may have synchronous appendiceal neoplasia.
  • PURPOSE: An individual with colorectal cancer has a 3 percent risk of synchronous colonic neoplasia and further 2 to 3 percent risk of metachronous cancer, a risk that has prompted colonic surveillance.
  • The appendix has a similar mucosal pattern to the colon and it has been hypothesized that appendicular adenocarcinoma may account for 1 percent of all colorectal malignancies.
  • A special interest of the senior author in appendiceal and rectal cancer has prompted routine removal of the appendix in all cases undergoing surgery for colorectal cancer.
  • RESULTS: In total, 169 patients under the care of a single surgeon had colorectal cancer resection between April 2002 and April 2005: 63 patients had right hemicolectomy, 29 had left hemicolectomy, and 77 had rectal cancer resection.
  • Seven of 169 appendices had abnormalities: 3 mucinous cystadenomas, 2 cystadenocarcinomas, 1 carcinoid tumor, and 1 villous adenoma.
  • Metachronous neoplasia is a risk in the retained appendix in patients with colorectal cancer.
  • Routine postoperative surveillance cannot assess the appendiceal mucosa, so there is little justification for not taking the opportunity to eliminate the possibility of future appendicitis or neoplasia.
  • [MeSH-major] Adenocarcinoma / epidemiology. Appendiceal Neoplasms / epidemiology. Colorectal Neoplasms / epidemiology. Neoplasms, Multiple Primary / epidemiology

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  • (PMID = 17763906.001).
  • [ISSN] 0012-3706
  • [Journal-full-title] Diseases of the colon and rectum
  • [ISO-abbreviation] Dis. Colon Rectum
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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35. Klöppel G, Anlauf M: Epidemiology, tumour biology and histopathological classification of neuroendocrine tumours of the gastrointestinal tract. Best Pract Res Clin Gastroenterol; 2005 Aug;19(4):507-17
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  • [Title] Epidemiology, tumour biology and histopathological classification of neuroendocrine tumours of the gastrointestinal tract.
  • The diffuse neuroendocrine cell gives rise to a heterogeneous population of tumours which differ in their morphological and functional features.
  • The term 'carcinoid', although well established in medical terminology, is therefore no longer adequate to cover the entire spectrum of neuroendocrine neoplasms.
  • Here we use the term neuroendocrine tumours (NET), which was suggested in the WHO classification of 2000, and review the most important NET entities that are currently recognised in the gastrointestinal tract, highlighting their distinguishing features.
  • [MeSH-major] Carcinoma, Neuroendocrine / pathology. Gastrointestinal Neoplasms / pathology
  • [MeSH-minor] Appendiceal Neoplasms / pathology. Cell Differentiation. Colonic Neoplasms / pathology. Esophageal Neoplasms / pathology. Humans. Ileal Neoplasms / pathology. Jejunal Neoplasms / pathology. Multiple Endocrine Neoplasia Type 1 / epidemiology. Multiple Endocrine Neoplasia Type 1 / pathology. Neoplasm Invasiveness. Prognosis. Rectal Neoplasms / pathology. Somatostatinoma / pathology. Stomach Neoplasms / pathology

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  • (PMID = 16183524.001).
  • [ISSN] 1521-6918
  • [Journal-full-title] Best practice & research. Clinical gastroenterology
  • [ISO-abbreviation] Best Pract Res Clin Gastroenterol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 43
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36. Rossi G, Nannini N, Bertolini F, Mengoli MC, Fano R, Cavazza A: Clear cell carcinoid of the appendix: an uncommon variant of lipid-rich neuroendocrine tumor with a broad differential diagnosis. Endocr Pathol; 2010 Dec;21(4):258-62
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  • [Title] Clear cell carcinoid of the appendix: an uncommon variant of lipid-rich neuroendocrine tumor with a broad differential diagnosis.
  • The designation of clear cell/lipid-rich refers to an unusual variant of neuroendocrine tumor ("carcinoid") described in several organs, but only recently observed in the appendix.
  • In this study, we report the morphologic, immunohistochemical, and ultrastructural features of an incidentally discovered appendiceal clear cell/lipid-rich carcinoid in a 32-year-old man without any evidence of von Hippel-Lindau disease.
  • Differential diagnosis with mimicking neoplastic and non-tumor lesions, epidemiology, and clinical behavior of this exceedingly rare variant of carcinoid of the appendix are also discussed.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Diagnosis, Differential. Humans. Immunohistochemistry. Male. Microscopy, Electron, Transmission

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  • (PMID = 20814762.001).
  • [ISSN] 1559-0097
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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37. Yang WL, Yan CQ, Wang FJ, Wang HL: [Diagnosis and surgical treatment of carcinoid tumors of the appendix in 64 patients]. Zhonghua Zhong Liu Za Zhi; 2008 Jul;30(7):538-40
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  • [Title] [Diagnosis and surgical treatment of carcinoid tumors of the appendix in 64 patients].
  • OBJECTIVE: To summarize the experience in the diagnosis and surgical treatment of carcinoid tumors of the appendix.
  • METHODS: From 1972 to 2006, 64 patients with carcinoid tumors of the appendix received surgical treatment in our hospitals.
  • RESULTS: Of the 64 cases, only 6 cases (9.4%) were correctly diagnosed preoperatively, while 58 (90.6%) not confirmed, with a misdiagnosis rate of 90.6%.
  • All patients underwent surgical treatment, including appendectomy in 54, ileocecectomy in 4, right hemicolectomy in 2 and right hemicolectomy with regional lymph node dissection in 4 cases.
  • The operation modes were determined according to the doctor's judgments based on the age of the patients, the nature, size, location, infiltration depth and lymph node metastasis of the tumors.
  • CONCLUSION: Carcinoid tumor of the appendix is rare with a high rate of misdiagnosis before operation.
  • Surgical resection is the only effective treatment for this disease and proper operation mode is the key to achieve good survival.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Appendiceal Neoplasms / surgery. Carcinoid Tumor / diagnosis. Carcinoid Tumor / surgery. Diagnostic Errors
  • [MeSH-minor] Adolescent. Adult. Aged. Appendectomy. Colectomy / methods. Female. Follow-Up Studies. Humans. Liver Neoplasms / secondary. Lymph Node Excision. Lymphatic Metastasis. Male. Middle Aged. Retrospective Studies. Survival Rate. Young Adult

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  • (PMID = 19062724.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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38. Benedix F, Reimer A, Gastinger I, Mroczkowski P, Lippert H, Kube R, Study Group Colon/Rectum Carcinoma Primary Tumor: Primary appendiceal carcinoma--epidemiology, surgery and survival: results of a German multi-center study. Eur J Surg Oncol; 2010 Aug;36(8):763-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary appendiceal carcinoma--epidemiology, surgery and survival: results of a German multi-center study.
  • BACKGROUND: While carcinoma of the colon is a common malignancy, primary carcinoma of the appendix is rare.
  • Many retrospective reviews outlined experience from different centers on appendiceal neoplasms.
  • The aim of this study was to analyze the type of surgery and survival of patients with appendiceal malignancies using data from a German multi-center observational study (31 341 patients).
  • METHODS: During a five-year period, 196 consecutive patients with malignant appendiceal tumors were distributed into four groups: appendiceal carcinoids, adenocarcinoma, mucinous adenocarcinoma and adenosquamous carcinoma.
  • The following parameters were analyzed: demographics, clinical presentation, comorbidities, type and appropriateness of surgery, final pathology and survival.
  • RESULTS: Adenocarcinoma had the highest incidence (50.5%).
  • Mean age at presentation was youngest for carcinoid tumors.
  • Carcinoid tumors had lowest tumor size and localized disease was present in 72.9%.
  • Overall 5-year survival was 83.1% for carcinoid vs. 49.2% for non-carcinoid tumors.
  • Histological subtype and tumor stage significantly affected survival.
  • CONCLUSIONS: Long-term outcome of carcinoid tumors is superior to non-carcinoid neoplasms.
  • Among all appendiceal neoplasms, adenosquamous carcinoma is the rarest histological subtype which is most commonly associated with advanced tumor stage and worst prognosis.
  • However, the high rate of right hemicolectomy in patients with small carcinoid tumors needs to be critically discussed.
  • [MeSH-major] Appendiceal Neoplasms / epidemiology. Appendiceal Neoplasms / surgery. Colectomy
  • [MeSH-minor] Adenocarcinoma / epidemiology. Adenocarcinoma / surgery. Adenocarcinoma, Mucinous / epidemiology. Adenocarcinoma, Mucinous / surgery. Adult. Aged. Appendicitis / diagnosis. Carcinoid Tumor / epidemiology. Carcinoid Tumor / surgery. Carcinoma, Adenosquamous / epidemiology. Carcinoma, Adenosquamous / surgery. Diagnosis, Differential. Female. Germany. Humans. Male. Middle Aged. Neoplasm Staging. Unnecessary Procedures / statistics & numerical data

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  • [Copyright] Copyright (c) 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 20561765.001).
  • [ISSN] 1532-2157
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
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39. Alsaad KO, Serra S, Schmitt A, Perren A, Chetty R: Cytokeratins 7 and 20 immunoexpression profile in goblet cell and classical carcinoids of appendix. Endocr Pathol; 2007;18(1):16-22
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  • [Title] Cytokeratins 7 and 20 immunoexpression profile in goblet cell and classical carcinoids of appendix.
  • Goblet cell carcinoid (GCC) of the vermiform appendix is an uncommon neoplasm and its histogenesis is controversial.
  • Whether GCC represents a morphological variant of classical appendiceal carcinoid or a mucin-producing adenocarcinoma is still conjectural.
  • Little is known about the immunohistochemical expression of cytokeratins 7 (CK7) and 20 (CK20) in appendiceal neuroendocrine tumors.
  • In this study, we compared the expression of CK7 and CK20 in 17 cases of appendiceal GCC and 25 cases of classical carcinoid.
  • The tumors were also evaluated for Ki-67 proliferation index, mitotic activity, tumor necrosis, extracellular pools of mucin, obvious intestinal type adenocarcinomatous foci, angiolymphatic permeation, perineural/neural infiltration, and the depth of invasion of the appendix wall.
  • The mitotic count ranged from 0 per 10 high power fields to 6 per 10 high power fields, with an average of 1.4 per 10 high power fields.
  • Immunohistochemically, all 17 (100%) of GCC exhibited strong and diffuse immunopositivity for CK20, whereas expression of CK7 was present in 12 cases (70.5%), ranging from 5 to 50% of tumor cells being labeled.
  • On the other hand, 25 cases of classical carcinoid tumors were consistently negative for CK7; however, 4 cases (16%) showed immunolabeling for CK20 in 25-50% of the tumor cells.
  • The Ki-67 labeling index in classical carcinoids ranged from 0 to 5%.
  • This study shows that in addition to the morphological differences, GCC (CK7/CK20-positive) and classical carcinoid (CK7/CK20-negative) differ in their expression of CK7 and CK20.
  • Goblet cell carcinoid should be regarded as a crypt cell or an amphicrine carcinoma rather than a variant of carcinoid tumor, a lesion that has benign connotations.
  • [MeSH-major] Appendiceal Neoplasms / metabolism. Carcinoid Tumor / metabolism. Goblet Cells / metabolism. Keratin-20 / metabolism. Keratin-7 / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Cell Proliferation. Female. Humans. Immunohistochemistry. Ki-67 Antigen / metabolism. Male. Middle Aged. Treatment Outcome

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  • (PMID = 17652796.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Keratin-20; 0 / Keratin-7; 0 / Ki-67 Antigen
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40. Oge T, Zeck W, Tamussino K: An adenocarcinoid tumour of the appendix mimicking advanced ovarian carcinoma. J Obstet Gynaecol; 2009 Nov;29(8):780-1
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  • [Title] An adenocarcinoid tumour of the appendix mimicking advanced ovarian carcinoma.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / diagnosis. Carcinoma / diagnosis. Ovarian Neoplasms / diagnosis
  • [MeSH-minor] Appendix / pathology. Appendix / surgery. Diagnosis, Differential. Female. Humans. Middle Aged. Ovary / pathology. Treatment Outcome

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  • (PMID = 19821688.001).
  • [ISSN] 1364-6893
  • [Journal-full-title] Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology
  • [ISO-abbreviation] J Obstet Gynaecol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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41. Huang B, Di MJ, Liu HS, Qiu LL, Sun LJ, Xu JP: [Primary malignant tumor of the appendix: clinicopathological analysis of 22 cases]. Zhejiang Da Xue Xue Bao Yi Xue Ban; 2009 Mar;38(2):194-8
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  • [Title] [Primary malignant tumor of the appendix: clinicopathological analysis of 22 cases].
  • OBJECTIVE: To investigate the clinicopathological and immunohistochemical features of primary malignant tumor of the appendix.
  • METHODS: The clinical data were reviewed; and histopathological and immunohistochemical features were analyzed in 22 cases with primary malignant tumor of the appendix.
  • RESULT: In 22 cases of primary malignant tumor of the appendix, 19 cases were carcinoid and 3 were adenocarcinoma.
  • Immunohistochemistry showed that the carcinoid was positively reacted to the neuroendocrine markers, and the adenocarcinoma was negatively reacted to the neuroendocrine markers.
  • CONCLUSION: Immunohistochemistry is useful in diagnosis of primary malignant tumor of the appendix, a rare type of cancer.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology

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  • (PMID = 19363829.001).
  • [ISSN] 1008-9292
  • [Journal-full-title] Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences
  • [ISO-abbreviation] Zhejiang Da Xue Xue Bao Yi Xue Ban
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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42. Iwuagwu OC, Jameel JK, Drew PJ, Hartley JE, Monson JR: Primary carcinoma of the appendix - Hull series. Dig Surg; 2005;22(3):163-7
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  • [Title] Primary carcinoma of the appendix - Hull series.
  • BACKGROUND: Appendiceal carcinoma (AC) is a rare entity that does not have a well-defined treatment strategy.
  • Once the diagnosis of AC is made, patients are treated by various strategies including surgery, chemotherapy depending on nodal status of the disease.
  • The histopathology reports of all appendiceal specimens removed were traced.
  • We did not include patients with primary carcinoid tumours or secondary adenocarcinoma.
  • RESULTS: There was an equal sex distribution.
  • [MeSH-major] Adenocarcinoma, Mucinous / surgery. Appendiceal Neoplasms / surgery

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  • [Copyright] Copyright (c) 2005 S. Karger AG, Basel.
  • (PMID = 16037676.001).
  • [ISSN] 0253-4886
  • [Journal-full-title] Digestive surgery
  • [ISO-abbreviation] Dig Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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43. O'Donnell ME, Badger SA, Beattie GC, Carson J, Garstin WI: Malignant neoplasms of the appendix. Int J Colorectal Dis; 2007 Oct;22(10):1239-48
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant neoplasms of the appendix.
  • BACKGROUND: Appendiceal neoplasms, first described in 1882, are still rare, with pre-operative diagnosis invariably difficult.
  • MATERIALS AND METHODS: A retrospective histopathological review of all appendicectomy specimens was completed between April 1994 and December 2003 to identify patients diagnosed with malignant neoplasms.
  • A literature search of the PubMed database was then performed using the medical search headings; appendix, tumour, neoplasm and malignancy.
  • RESULTS: Twenty-two patients (eight men) were identified during the study period, with no age difference between gender (mean age in women 58, range 14-83 vs mean age in men 55, range 16-78).
  • Eleven patients were found to have carcinoid-type tumours, eight patients with adenocarcinomas and three patients with lymphomas.
  • Other appendiceal pathologies were identified after appendicectomies, hemicolectomy and oophorectomy.
  • Patients with classical carcinoid tumours (CCT) had better outcomes than patients with the goblet cell carcinoid, adenocarcinoma and lymphoma.
  • CONCLUSIONS: From our own experience and a subsequent review of the literature, we recommend right hemicolectomy as the treatment of choice for all malignant appendiceal neoplasms, except for small CCT less than 2 cm in diameter at the tip of the appendix, with a low proliferative index, without angiolymphatic or mesoappendiceal extension.
  • Further adjuvant therapy should be considered after oncological assessment.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Appendiceal Neoplasms / therapy

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  • (PMID = 17447078.001).
  • [ISSN] 0179-1958
  • [Journal-full-title] International journal of colorectal disease
  • [ISO-abbreviation] Int J Colorectal Dis
  • [Language] eng
  • [Publication-type] Journal Article; Meta-Analysis
  • [Publication-country] Germany
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44. Louzi A, Nargis Y, Belaabidia B, Alatawna H, Benelkhaiat R, Finech B, Dafali AE: [Appendicular mucocele caused by carcinoid tumor during pregnancy]. Gynecol Obstet Fertil; 2006 Nov;34(11):1051-4
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  • [Title] [Appendicular mucocele caused by carcinoid tumor during pregnancy].
  • [Transliterated title] Mucocèle de l'appendice sur tumeur carcinoïde pendant la grossesse.
  • Mucocele of the appendix secondary to appendicular carcinoid tumour is a rare entity.
  • The interaction between carcinoid tumour or mucocele of the appendix and pregnancy is not yet elucidated.
  • Prognosis depends on the size of the carcinoid tumour and its regional invasion.
  • We report a case of appendicular mucocele caused by a carcinoid tumour in a 36-year-old pregnant woman who had been admitted because of acute appendicitis.
  • Pathological examination of the appendicectomy specimen revealed a carcinoid tumour measuring 2 cm.
  • [MeSH-major] Appendiceal Neoplasms / complications. Carcinoid Tumor / complications. Mucocele / etiology. Pregnancy Complications, Neoplastic / surgery


45. Coursey CA, Nelson RC, Moreno RD, Dodd LG, Patel MB, Vaslef S: Carcinoid tumors of the appendix: are these tumors identifiable prospectively on preoperative CT? Am Surg; 2010 Mar;76(3):273-5
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  • [Title] Carcinoid tumors of the appendix: are these tumors identifiable prospectively on preoperative CT?
  • The purpose of this study was to determine if carcinoid tumors of the appendix were identified prospectively on preoperative CT at our institution during the last decade.
  • A surgical database search performed using the Current Procedural Terminology codes for appendectomy and colectomy yielded 2108 patients who underwent appendectomy or colectomy with removal of the appendix from January 1998 through September 2007.
  • Pathology reports were reviewed to identify patients in whom an appendiceal carcinoid tumor was identified.
  • Twenty-three carcinoid tumors (1.1%; 15 women [65.2%], eight men [34.8%]; average age 54 years [range, 23 to 86 years]) were identified.
  • No tumors were identified prospectively on CT.
  • Average reported tumor size was 6.1 mm (range, 1.5 to 15 mm; n = 18).
  • A tip or distal location was reported for all tumors for which a location was given (n = 15).
  • Carcinoid tumors occurred in 1.1 per cent of appendix specimens.
  • These tumors were all less than or 1.5 cm in size.
  • Likely as a result of their small size, none of these tumors was identified prospectively on preoperative CT.
  • [MeSH-major] Appendiceal Neoplasms / radiography. Carcinoid Tumor / radiography. Tomography, X-Ray Computed
  • [MeSH-minor] Adenocarcinoma / epidemiology. Adenocarcinoma / surgery. Adult. Aged. Aged, 80 and over. Female. Humans. Incidental Findings. Intestinal Neoplasms / epidemiology. Intestinal Neoplasms / surgery. Male. Middle Aged. Neoplasms, Multiple Primary / surgery. Preoperative Period. Young Adult

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  • (PMID = 20349655.001).
  • [ISSN] 0003-1348
  • [Journal-full-title] The American surgeon
  • [ISO-abbreviation] Am Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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46. Feo CF, Porcu A, Scanu AM, Ginesu GC, Fancellu A, Lorettu A, Dettori G: Primary appendiceal tumors: report on 10 cases. Int Surg; 2009 Jul-Sep;94(3):224-7
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  • [Title] Primary appendiceal tumors: report on 10 cases.
  • We report our experience on 10 patients with primary tumors of the appendix treated at our institution from 1998 to 2005.
  • Six tumors were malignant, and the remaining were benign.
  • Two of the four patients with benign tumors died from causes unrelated to the appendiceal neoplasm.
  • The 6 patients with malignant tumors and the other 2 with benign disease were alive and disease free after a mean follow-up of 43 months.
  • Despite the rarity of appendiceal primary tumors, surgeons should be aware of these neoplasms for making correct treatment decisions.
  • We stress the importance of laparoscopic exploration in the management of appendiceal masses.
  • [MeSH-major] Adenocarcinoma / surgery. Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery

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  • (PMID = 20187516.001).
  • [ISSN] 0020-8868
  • [Journal-full-title] International surgery
  • [ISO-abbreviation] Int Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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47. Dang D, DeRenne LA: Goblet Cell Carcinoid Presenting as Appendicitis 1.5 Years After a Normal CT Scan. Radiol Case Rep; 2007;2(2):69-71
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  • [Title] Goblet Cell Carcinoid Presenting as Appendicitis 1.5 Years After a Normal CT Scan.
  • A 60-year-old man presented with signs and symptoms of acute appendicitis and was found to have goblet cell carcinoid (GCC) of the appendix.
  • The patient had no evidence of appendiceal disease on a computed tomography study one and a half year before.
  • Due to the rarity of this tumor, previous case reports and series did not discuss the time course of the tumor's onset.
  • This case report provides evidence that GCC can occur within a period of 1.5 years.
  • This case report also discusses relevant histopathological aspects of GCC as pertain to cross-sectional imaging findings and illustrates the importance of considering other types of appendiceal disease in the diagnosis of a patient presenting with acute appendicitis.

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  • (PMID = 27303467.001).
  • [ISSN] 1930-0433
  • [Journal-full-title] Radiology case reports
  • [ISO-abbreviation] Radiol Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  • [Other-IDs] NLM/ PMC4891629
  • [Keywords] NOTNLM ; CT, computed tomography
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48. Wang HL, Dhall D: Goblet or signet ring cells: that is the question. Adv Anat Pathol; 2009 Jul;16(4):247-54
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  • Goblet cell carcinoid tumor is a rare mixed endocrine-exocrine neoplasm of the appendix.
  • It carries an intermediate biologic behavior between a classic carcinoid tumor and a conventional adenocarcinoma.
  • A recent retrospective study of a large number of appendiceal goblet cell carcinoids has shown that these tumors can be stratified into 3 subgroups based on careful histologic analysis: typical goblet cell carcinoid (group A); adenocarcinoma ex goblet cell carcinoid, signet ring cell type (group B); and adenocarcinoma ex goblet cell carcinoid, poorly differentiated carcinoma type (group C).
  • Clinical follow-up data show that the histologic classification correlates with patient survival.
  • Thus, meticulous histologic evaluation is of critical importance in determining the prognosis and guiding the management of patients with goblet cell carcinoids.
  • This brief review summarizes these recent findings and raises a few issues that may need to be further addressed to better apply this classification to our practice.
  • [MeSH-major] Adenocarcinoma / pathology. Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Carcinoma, Signet Ring Cell / pathology. Goblet Cells / pathology

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  • (PMID = 19546612.001).
  • [ISSN] 1533-4031
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 11
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49. Bucher P, Gervaz P, Ris F, Oulhaci W, Egger JF, Morel P: Surgical treatment of appendiceal adenocarcinoid (goblet cell carcinoid). World J Surg; 2005 Nov;29(11):1436-9
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  • [Title] Surgical treatment of appendiceal adenocarcinoid (goblet cell carcinoid).
  • Adenocarcinoid of the appendix is an infrequent tumor with histologic features of both adenocarcinoma and carcinoid tumor.
  • Although its malignant potential remains unclear, adenocarcinoids seem to be biologically more aggressive than conventional carcinoids.
  • The aim of this study was to analyze long-term results of surgical treatment for appendiceal adenocarcinoid.
  • A retrospective review (1991-2003) identified seven patients (median age 72, range 27-81 years) treated for appendiceal adenocarcinoid.
  • Indications for colectomy were tumor size (three cases) associated with appendectomy margin invasion in one case.
  • One patient with lymph node and peritoneal involvement experienced recurrence 9 months after hemicolectomy and died of the disease at 2 years.
  • Five patients were alive without disease at the time of the last follow-up.
  • Our results suggest that appendectomy alone could be used for appendiceal adenocarcinoid provided that the tumor (1) is less than 1 cm;.
  • (2) does not extend beyond the appendix adventitia;.
  • (3) has less than 2 mitoses/10 high power fields; and (4) has surgical margins that are tumor free.
  • The risk for developing colorectal adenocarcinoma seems to be extremely high in patients treated for appendiceal adenocarcinoid and warrants close follow-up with colonoscopic screening.
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Colectomy. Female. Humans. Male. Middle Aged. Neoplasm Invasiveness. Retrospective Studies

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  • (PMID = 16136284.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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50. Couvelard A: [Appendicular pathology. Goblet cell carcinoid]. Ann Pathol; 2010 Apr;30(2):120-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Appendicular pathology. Goblet cell carcinoid].
  • [Transliterated title] Pathologie de l'appendice. Cas no 6. Carcinoïde à cellules caliciformes (adénocarcinoïde).
  • [MeSH-major] Appendiceal Neoplasms / pathology. Appendicitis / etiology. Appendix / pathology. Carcinoid Tumor / pathology
  • [MeSH-minor] Alcian Blue. Appendectomy. Biomarkers, Tumor / analysis. Chromogranin A / analysis. Coloring Agents. Female. Humans. Keratin-20 / analysis. Middle Aged. Mucin-2 / analysis. Neoplasm Invasiveness. Neoplasm Proteins / analysis. Staining and Labeling

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  • (PMID = 20451070.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CHGA protein, human; 0 / Chromogranin A; 0 / Coloring Agents; 0 / Keratin-20; 0 / MUC2 protein, human; 0 / Mucin-2; 0 / Neoplasm Proteins; P4448TJR7J / Alcian Blue
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51. Long KB, Srivastava A, Hirsch MS, Hornick JL: PAX8 Expression in well-differentiated pancreatic endocrine tumors: correlation with clinicopathologic features and comparison with gastrointestinal and pulmonary carcinoid tumors. Am J Surg Pathol; 2010 May;34(5):723-9
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  • [Title] PAX8 Expression in well-differentiated pancreatic endocrine tumors: correlation with clinicopathologic features and comparison with gastrointestinal and pulmonary carcinoid tumors.
  • However, PAX8 expression has not previously been examined in pancreatic endocrine tumors (PETs).
  • PAX8 expression in other well-differentiated neuroendocrine tumors (WDNETs) was also studied.
  • In total, 190 tumors were evaluated: 156 primary WDNETs (63 PETs, 31 ileal, 5 duodenal, 5 gastric, 19 appendiceal, 13 rectal, and 20 pulmonary carcinoid tumors) and 34 liver metastases (18 PETs and 16 ileal carcinoid tumors).
  • Expression of PAX8 was significantly associated with WHO category 1.1 ("benign" behavior) compared with category 1.2 (uncertain behavior) or 2 (well-differentiated endocrine carcinoma) (positive in 100%, 64%, and 52% of tumors, respectively; P<0.05).
  • PAX8-positive PETs were also significantly smaller and more often clinically functional; PAX8-negative tumors were more frequently associated with liver metastases.
  • PAX8 expression was detected in 0/20 (0%) pulmonary, 1/5 (20%) gastric, 5/5 (100%) duodenal, 0/31 (0%) ileal, 4/19 (21%) appendiceal, and 11/13 (85%) rectal carcinoid tumors.
  • Among the liver metastases, PAX8 was positive in 9/18 (50%) metastatic PETs compared with 0/16 (0%) metastatic ileal carcinoid tumors.
  • In the GI tract, PAX8 is positive in the majority of duodenal and rectal carcinoid tumors, and in a minor subset of appendiceal and gastric carcinoids.
  • PAX8 expression is absent in ileal and pulmonary carcinoid tumors.
  • PAX8 immunostaining may be helpful in determining the primary site for a WDNET metastatic to the liver, as ileal (PAX8 negative) and pancreatic (PAX8 positive) tumors most often present as a metastasis from an occult primary.
  • PAX8 may also be a prognostic marker in PETs, as loss of expression is associated with malignant behavior.
  • [MeSH-major] Adenoma, Islet Cell / pathology. Carcinoid Tumor / pathology. Carcinoma, Islet Cell / secondary. Gastrointestinal Neoplasms / pathology. Lung Neoplasms / pathology. Paired Box Transcription Factors / metabolism. Pancreatic Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Female. Humans. Immunohistochemistry. Islets of Langerhans / metabolism. Islets of Langerhans / pathology. Liver Neoplasms / metabolism. Liver Neoplasms / secondary. Lymph Nodes / pathology. Male. Middle Aged

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  • [CommentIn] Am J Surg Pathol. 2011 Dec;35(12):1906-8 [22067332.001]
  • (PMID = 20414099.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / PAX8 protein, human; 0 / Paired Box Transcription Factors
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52. Chetty R: Goblet cell carcinoid tumours of the appendix: a unique neuroendocrine tumour. Histopathology; 2008 May;52(6):770-1
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Goblet cell carcinoid tumours of the appendix: a unique neuroendocrine tumour.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology
  • [MeSH-minor] Appendix / pathology. Humans

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  • [CommentOn] Histopathology. 2007 Dec;51(6):763-73 [18042066.001]
  • (PMID = 18439158.001).
  • [ISSN] 1365-2559
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] England
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53. O'Donnell ME, Carson J, Garstin WI: Surgical treatment of malignant carcinoid tumours of the appendix. Int J Clin Pract; 2007 Mar;61(3):431-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical treatment of malignant carcinoid tumours of the appendix.
  • Since their first description in 1882, malignant neoplasms of the appendix still remain rare.
  • Malignant carcinoid tumours are the most common accounting for 85% of all appendiceal neoplasms.
  • Preoperative diagnosis is invariably difficult, and precise treatment protocols for these neoplasms remain unclear.
  • A retrospective review of all malignant carcinoid tumours diagnosed in our hospital between April 1994 and December 2003 was performed.
  • Nine patients were identified with classical carcinoid tumours (CCT); (M = 3 and F = 6, mean age: 43, range 14-81) and two patients with goblet-cell morphology (F = 2, age 46 and 76).
  • Other appendiceal pathologies were identified following hemicolectomy and oophorectomy.
  • CCT are the most common tumours and have the better prognosis.
  • From our experience and subsequent review of the literature, we recommend right hemicolectomy as the treatment of choice for malignant carcinoid tumours.
  • However, small CCTs less than 2 cm in diameter at the tip of the appendix, with a low proliferative index, without angiolymphatic or mesoappendiceal extension can be treated by appendicectomy.
  • Following oncological assessment, further adjuvant therapy should be considered for patients with advanced disease.
  • [MeSH-major] Appendectomy / methods. Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery. Colectomy / methods

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  • (PMID = 16911574.001).
  • [ISSN] 1368-5031
  • [Journal-full-title] International journal of clinical practice
  • [ISO-abbreviation] Int. J. Clin. Pract.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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54. Nikolaou A, Thomas D, Kampanellou C, Alexandraki K, Andersson LG, Sundin A, Kaltsas G: The value of 11C-5-hydroxy-tryptophan positron emission tomography in neuroendocrine tumor diagnosis and management: experience from one center. J Endocrinol Invest; 2010 Dec;33(11):794-9
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  • [Title] The value of 11C-5-hydroxy-tryptophan positron emission tomography in neuroendocrine tumor diagnosis and management: experience from one center.
  • Many neuroendocrine tumors (NET) are small and may escape localization by conventional imaging techniques.
  • Nine patients with clinically, biochemically and/or histologically confirmed NET and negative computerized tomography (CT) or magnetic resonance imaging (MRI), and 111In-pentetreotide (Octreoscan) scintigraphy underwent imaging with 11C-5-HTP-PET/CT in order to:.
  • 1) detect the primary tumor lesion in three patients;.
  • 2) detect residual disease in two patients with appendiceal carcinoid, one with rectal carcinoid, one with midgut carcinoid, and one with ectopic ACTH secretion (EAS) due to residual pulmonary carcinoid; and 3) restage a patient with medullary thyroid carcinoma (MTC) and hepatic metastases.
  • 11C-5-HTP-PET/CT detected lesions in the mediastinum in a patient with EAS due to a pulmonary carcinoid, further hepatic metastases in a patient with carcinoid syndrome (CS) from a NET of unknown primary, further hepatic metastases in the patient with MTC, and hepatic metastases in the patient with midgut carcinoid.
  • The 11C-5-HTP-PET/CT findings contributed to radical cure of the patient with recurrent EAS, and pointed towards bilateral adrenalectomy in the patient with EAS without evident primary tumor.
  • In addition, 11C-5- HTP-PET/CT directed towards combined surgical and medical treatment in the patient with CS and multiple rather than single hepatic metastases and in the patient with midgut carcinoid, and towards continuation of medical treatment in the patient with MTC.
  • 11C-5-HTP-PET/CT is a useful imaging technique, providing additional information for the diagnosis, staging and decision-making regarding management of patients with NET.
  • [MeSH-major] 5-Hydroxytryptophan. Carbon Radioisotopes. Neuroendocrine Tumors / diagnostic imaging. Radiopharmaceuticals
  • [MeSH-minor] ACTH Syndrome, Ectopic / diagnostic imaging. Adult. Carcinoid Tumor / diagnostic imaging. Female. Humans. Male. Middle Aged. Neoplasm, Residual / diagnostic imaging. Positron-Emission Tomography / methods

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  • (PMID = 20332708.001).
  • [ISSN] 1720-8386
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Carbon Radioisotopes; 0 / Radiopharmaceuticals; C1LJO185Q9 / 5-Hydroxytryptophan
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55. Kunz J, Makek M: [Primary adenocarcinoma of the appendix as differential diagnosis of advanced ovarian carcinoma]. Praxis (Bern 1994); 2006 Aug 16;95(33):1217-25
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  • [Title] [Primary adenocarcinoma of the appendix as differential diagnosis of advanced ovarian carcinoma].
  • [Transliterated title] Das primäre Adenokarzinom der Appendix als Differentialdiagnose des fortgeschrittenen Ovarialkarzinoms.
  • Malignant tumours of the appendix are rare.
  • They are usually carcinoid tumours that must be distinguished from extremely rare adenocarcinomas.
  • Metastatic mucinous adenocarcinomas of the appendix are only reported as case histories.
  • In clinical terms, the tumours usually manifest themselves as acute appendicitis, as ruptured appendicitis, as a tumour in the right lower abdominal quadrant or as a pelvic tumour, which are generally mistaken for an ovarian tumour with the same sonographic image.
  • Advanced primary adenocarcinomas of the appendix with ovarian metastases cannot be distinguished intraoperatively from a FIGO III ovarian carcinoma.
  • Surgical therapy of the isolated primary appendiceal carcinoma consists of a hemicolectomy--an appendectomy in favourable cases--and, in the case of a metastasised carcinoma, according to the guidelines for an advanced ovarian or colon carcinoma.
  • [MeSH-major] Adenocarcinoma, Mucinous / diagnosis. Adenocarcinoma, Mucinous / secondary. Appendiceal Neoplasms / diagnosis. Carcinoma, Signet Ring Cell / diagnosis. Carcinoma, Signet Ring Cell / secondary. Ovarian Neoplasms / diagnosis. Ovarian Neoplasms / secondary
  • [MeSH-minor] Appendix / pathology. Appendix / surgery. Diagnosis, Differential. Female. Humans. Hysterectomy, Vaginal. Middle Aged. Omentum / pathology. Omentum / surgery. Ovary / pathology. Ovary / surgery. Peritoneal Neoplasms / diagnosis. Peritoneal Neoplasms / pathology. Peritoneal Neoplasms / secondary. Peritoneal Neoplasms / surgery. Peritoneum / pathology. Peritoneum / surgery. Postoperative Complications / diagnosis. Postoperative Complications / pathology. Postoperative Complications / surgery. Reoperation

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  • (PMID = 16939122.001).
  • [ISSN] 1661-8157
  • [Journal-full-title] Praxis
  • [ISO-abbreviation] Praxis (Bern 1994)
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Switzerland
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56. Fornaro R, Picori E, Stabilini C, Frascio M, Sticchi C, Boccardo C, Ricci B, Giannetta E: [Carcinoid tumors of the appendix: when right colectomy?]. G Chir; 2006 May;27(5):233-9
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  • [Title] [Carcinoid tumors of the appendix: when right colectomy?].
  • [Transliterated title] I tumori carcinoidi dell'appendice. Quando l'emicolectomia destra?
  • Three cases of carcinoid tumour of the appendix (about 0,3 % of all performed appendectomies) has induced the Authors to a review of the literature with the aim to underline the most important biological and pathological findings and the current clinic and therapeutic knowledges.
  • The kind of surgical intervention, that is the entity of the surgical demolition, for the treatment of the carcinoid tumours of the appendix is still controversial: appendectomy or right colectomy?
  • It is possible identify, also during the operation for an appendicitis or for other abdominal lesions, criteria that can orient toward a major surgery (size of the neoplasia, subserosal lymphatic invasion, infiltration of the serosa, diffusion in the meso-appendix, location in closeness of the base of the appendix, invasion of the the locoregional lymph nodes, presence of metastases, section ?margins, number of mitoses, cellular pleiomorfism).
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery. Colectomy

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  • (PMID = 16857114.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] ita
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 31
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57. Smaldone GM, Richard SD, Krivak TC, Kelley JL 3rd, Edwards RP: Pregnancy after tumor debulking and intraperitoneal cisplatin for appendiceal carcinoid tumor. Obstet Gynecol; 2007 Aug;110(2 Pt 2):477-9
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  • [Title] Pregnancy after tumor debulking and intraperitoneal cisplatin for appendiceal carcinoid tumor.
  • CASE: We present the case of a 25-year-old woman with appendiceal carcinoid tumor treated with intraperitoneal cisplatin for peritoneal recurrence after a fertility-sparing cytoreductive procedure.
  • She subsequently had a successful second pregnancy and is currently alive without evidence of her disease.
  • CONCLUSION: Conception is possible after tumor debulking and intraperitoneal chemotherapy.
  • [MeSH-major] Antineoplastic Agents / adverse effects. Appendiceal Neoplasms / drug therapy. Carcinoid Tumor / drug therapy. Cisplatin / adverse effects. Infertility, Female / therapy. Neoplasm Recurrence, Local / drug therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Female. Humans. Infusions, Parenteral. Pregnancy. Pregnancy Complications, Neoplastic. Pregnancy Outcome. Reproductive Techniques, Assisted


58. van Eeden S, Offerhaus GJ, Hart AA, Boerrigter L, Nederlof PM, Porter E, van Velthuysen ML: Goblet cell carcinoid of the appendix: a specific type of carcinoma. Histopathology; 2007 Dec;51(6):763-73
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  • [Title] Goblet cell carcinoid of the appendix: a specific type of carcinoma.
  • AIMS: Goblet cell carcinoid is a poorly understood tumour of the appendix.
  • The aim of this study was to determine whether it should be regarded as a separate entity or as a variant of classical carcinoid.
  • METHODS AND RESULTS: The immunohistochemical expression pattern of 21 markers and the mutation status of KRas codon 12 were determined in 16 goblet cell carcinoids and compared with 14 classical carcinoids, 19 colonic adenocarcinomas and 10 appendiceal mucinous cystadeno (carcino)mas.
  • The results were subjected to a stepwise linear discriminant analysis.
  • Goblet cell carcinoids were significantly different from the control groups.
  • The most important markers for discriminating between the groups were CEA (classical carcinoid versus all others), KRas mutation (present in all mucinous cystadeno (carcino)mas), beta-catenin (goblet cell carcinoid versus left sided colonic adenocarcinoma) and chromogranin (goblet cell carcinoid versus right sided colonic adenocarcinoma).
  • Expression of Math1 and HD5 was similar in goblet cell carcinoid and colonic adenocarcinoma but absent in classical carcinoid.
  • CONCLUSION: The results suggest that goblet cell carcinoids should be regarded as a separate entity.
  • The formerly used term 'crypt cell carcinoma' may be more appropriate because it reflects the more aggressive clinical behaviour of these tumours as well as their greater similarity to adenocarcinomas rather than to carcinoids.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Biomarkers, Tumor / analysis. Carcinoid Tumor / pathology

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  • [CommentIn] Histopathology. 2008 May;52(6):770-1 [18439158.001]
  • (PMID = 18042066.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Grant] United States / NIMHD NIH HHS / MD / 1P20 MD001824
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / KRAS protein, human; 0 / Proto-Oncogene Proteins; EC 3.6.5.2 / ras Proteins
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59. Toumpanakis C, Standish RA, Baishnab E, Winslet MC, Caplin ME: Goblet cell carcinoid tumors (adenocarcinoid) of the appendix. Dis Colon Rectum; 2007 Mar;50(3):315-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Goblet cell carcinoid tumors (adenocarcinoid) of the appendix.
  • PURPOSE: Goblet cell appendiceal carcinoids represent rare tumors that exhibit histologic features of both adenocarcinomas and neuroendocrine tumors.
  • We present the long-term results of a series of 15 patients, focusing on clinical manifestations, diagnosis, and management.
  • RESULTS: The majority of patients (7/15) presented with symptoms compatible with acute appendicitis.
  • Right hemicolectomy was performed in all except one, who subsequently developed metastases.
  • Ki67 index was greater than 20 percent in all of them, while in only one with local tumor.
  • Combination chemotherapy with either cisplatin plus etoposide or with 5-fluorouracil, cisplatin, and streptozotocin was administered to all patients with metastases resulting in temporary stabilization of disease.
  • Twelve patients are alive, while three died of their disease 9, 13, and 14 months after diagnosis.
  • CONCLUSIONS: The diagnostic value of chromogranin-A, urinary 5-hydroxy-indol-acetic acid, and (111)Indium-labeled octreotide scintigraphy seems to be limited in these tumors.
  • Ki67 index appears to predict tumor behavior.
  • Chemotherapy may have efficacy in metastatic disease, however, more data are required to determine this and the optimal regimen.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / diagnosis
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Appendectomy. Biomarkers, Tumor / analysis. Cisplatin / administration & dosage. Colectomy. Combined Modality Therapy. Diagnosis, Differential. Etoposide / administration & dosage. Female. Fluorouracil / administration & dosage. Humans. Immunohistochemistry. Ki-67 Antigen / analysis. Male. Middle Aged. Neoplasm Metastasis. Retrospective Studies. Streptozocin / administration & dosage


60. Louthan O: [Neuroendocrine tumours of the appendix]. Vnitr Lek; 2009 Nov;55(11):1051-5
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  • [Title] [Neuroendocrine tumours of the appendix].
  • [Transliterated title] Neuroendokrinní tumory appendixu.
  • According to WHO, neuroendocrine tumors of the appendix (appendiceal carcinoids) are defined as 1. well-differentiated endocrine tumors with benign or uncertain behavior, 2. well-differentiated endocrine carcinoma and 3. goblet cell carcinoma.
  • These tumors are usually diagnosed incidentally during appendectomy.
  • Carcinoid syndrome is rare in appendiceal carcinoid.
  • Tumor size greater than 2 cm is the most important parameter for prognosis.
  • Most patients are cured by appendectomy (appendiceal tumors < or = 2 cm), tumors with a diameter > 2 cm should be managed by right hemicolectomy.
  • [MeSH-major] Appendiceal Neoplasms. Neuroendocrine Tumors

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  • (PMID = 20017436.001).
  • [ISSN] 0042-773X
  • [Journal-full-title] Vnitr̆ní lékar̆ství
  • [ISO-abbreviation] Vnitr Lek
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 26
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61. Cadden I, Johnston BT, Turner G, McCance D, Ardill J, McGinty A: An evaluation of cyclooxygenase-2 as a prognostic biomarker in mid-gut carcinoid tumours. Neuroendocrinology; 2007;86(2):104-11
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  • [Title] An evaluation of cyclooxygenase-2 as a prognostic biomarker in mid-gut carcinoid tumours.
  • BACKGROUND/AIMS: Mid-gut carcinoids (MGC) are the most common of the gastrointestinal carcinoid tumours.
  • Cox-2 and Bcl-2 were evaluated as prognostic biomarkers in a cohort of well-characterised non-appendiceal MGC.
  • METHODS: Tissue from the primary MGC tumours of 37 patients was subjected to immunohistochemical detection of Cox-2 and Bcl-2.
  • The study assessed whether tumour-associated Cox-2 and Bcl-2 expression were related to patient survival.
  • RESULTS: Cox-2 expression was demonstrated in 30/36 primary tumours.
  • When all tumours were analysed, Cox regression analysis indicated a trend towards worsening survival with increasing Cox-2 histoscore (intensity x proportion; hazard ratio 1.53, 95% CI 0.93, 2.52; p = 0.09).
  • Analysis of Cox-2-positive tumours revealed a highly significant association between increasing histoscore and decreased survival (hazard ratio 3.03, 95% CI 1.33, 6.91; p = 0.008).
  • Tumour-associated Bcl-2 expression had no effect on patient survival (hazard ratio 1.12, 95% CI 0.42, 2.99; p = 0.82).
  • Analysis of the Cox-2 histoscores of primary tumours and their corresponding secondary lesions revealed a statistically significant trend towards increasing histoscore in the latter (Wilcoxon p = 0.04).
  • [MeSH-major] Biomarkers, Tumor / metabolism. Carcinoid Tumor / metabolism. Carcinoid Tumor / pathology. Cyclooxygenase 2 / metabolism. Intestinal Neoplasms / metabolism. Intestinal Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Appendix / pathology. Colon / pathology. Female. Humans. Intestine, Small / pathology. Liver Neoplasms / metabolism. Liver Neoplasms / secondary. Male. Middle Aged. Prognosis. Proportional Hazards Models. Proto-Oncogene Proteins c-bcl-2 / metabolism

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  • [Copyright] (c) 2007 S. Karger AG, Basel.
  • (PMID = 17700013.001).
  • [ISSN] 1423-0194
  • [Journal-full-title] Neuroendocrinology
  • [ISO-abbreviation] Neuroendocrinology
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Proto-Oncogene Proteins c-bcl-2; EC 1.14.99.1 / Cyclooxygenase 2
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62. Paredes Esteban RM, Martínez de Vitoria JM, García Ruiz M: [An association of mucocele and carcinoid tumour of the appendix]. Cir Pediatr; 2006 Oct;19(4):250-2
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  • [Title] [An association of mucocele and carcinoid tumour of the appendix].
  • [Transliterated title] Un caso de asociación de mucocele y tumor carcinoide apendicular.
  • The carcinoid tumour of the appendix and the mucocele are entities rather infrequent in children and are normally diagnosed in the course of an appendicectomy that is, in most of the cases, curative.
  • The authors present a case of carcinoid tumour of the appendix associated to mucocele in a male, aged 10 patient, who underwent an appendicectomy by suspicion of an acute appendicitis.
  • The histologic study confirmed the presence of a mucocele of the appendix associated to a carcinoid tumour with a 0,7 cm diameter.
  • The serum serotonin and chromogranin A assessment was normal, and the TAC and colonoscopic examination resulted in no meaningful findings.
  • The appendicectomy itself was curative, deeming it the appropiated treatment in tumours under 2 cm diameter.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Mucocele / pathology
  • [MeSH-minor] Appendectomy. Appendix / pathology. Appendix / surgery. Child. Humans. Male. Treatment Outcome

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  • (PMID = 17352118.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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63. Hadj-Taieb I, Masmoudi A, Ayadi L, Meziou TJ, Khabir A, Charfeddine A, Boujelbène S, Beyrouti MI, Tahri N, Boudawara T, Turki H, Zahaf A: [Appendicular cystadenocarcinoma with cutaneous fistula]. Ann Dermatol Venereol; 2010 Mar;137(3):198-202
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  • [Transliterated title] Cystadénocarcinome appendiculaire fistulisé à la peau.
  • We report a case of fistular lesions of the buttocks revealing a mixed tumour of the appendix involving mucinous cystadenocarcinoma and carcinoid tumour.
  • Histological examination of skin biopsy specimens identified infiltration of the dermis by metastatic mucinous adenocarcinoma while colonoscopy showed a caecal tumour measuring 4 cm.
  • Histopathological examination of surgical specimen confirmed mixed tumour consisting of perforated mucinous cystadenocarcinoma and carcinoid tumour of the appendix.
  • DISCUSSION: Cutaneous metastasis of colorectal cancer is an uncommon event that usually occurs after identification of the primary tumour and generally indicates advanced-stage disease and an ominous prognosis.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Cecal Neoplasms / pathology. Cutaneous Fistula / etiology. Cystadenocarcinoma, Mucinous / pathology. Neoplasms, Multiple Primary / pathology
  • [MeSH-minor] Aged. Carcinoid Tumor / pathology. Female. Humans. Skin Neoplasms / secondary

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  • [Copyright] Copyright 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20227562.001).
  • [ISSN] 0151-9638
  • [Journal-full-title] Annales de dermatologie et de vénéréologie
  • [ISO-abbreviation] Ann Dermatol Venereol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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64. Zafar S, Chen H, Sun W, Das K: Cytology of metastatic appendiceal goblet cell carcinoid in pleural effusion fluid: a case report. Diagn Cytopathol; 2008 Dec;36(12):894-8
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  • [Title] Cytology of metastatic appendiceal goblet cell carcinoid in pleural effusion fluid: a case report.
  • Goblet cell appendiceal carcinoid (GCAC) is a rare neoplasm.
  • We described the case of a 72-year-old man who presented with symptoms related to ascites.
  • A computed tomography (CT) scan showed a mass involving the base of the appendix, ascites and concomitant pleural effusion.
  • The appendiceal mass was biopsied and diagnosed as a GCAC.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / diagnosis. Pleural Effusion, Malignant / diagnosis

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18855885.001).
  • [ISSN] 1097-0339
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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65. Tchana-Sato V, Detry O, Polus M, Thiry A, Detroz B, Maweja S, Hamoir E, Defechereux T, Coimbra C, De Roover A, Meurisse M, Honoré P: Carcinoid tumor of the appendix: a consecutive series from 1237 appendectomies. World J Gastroenterol; 2006 Nov 7;12(41):6699-701
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Carcinoid tumor of the appendix: a consecutive series from 1237 appendectomies.
  • AIM: To report the experience of the CHU Sart Tilman, University of Liege, Belgium, in the management of appendiceal carinoid tumor.
  • RESULTS: Among the 1237 appendectomies, 5 appendiceal carcinoid tumors were identified (0.4%) in 4 male and 1 female patients, with a mean age of 29.2 years (range: 6-82 years).
  • All tumors were located at the tip of the appendix with a mean diameter of 0.6 cm (range: 0.3-1.0 cm).
  • All patients were alive and disease-free during a mean follow-up of 33 mo.
  • CONCLUSION: Appendiceal carcinoid tumor most often presents as appendicitis.
  • Appendiceal carcinoid tumor can be managed by simple appendectomy and resection of the mesoappendix, if its size is <or=1 cm.
  • [MeSH-major] Appendectomy / methods. Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery

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  • (PMID = 17075987.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] China
  • [Number-of-references] 29
  • [Other-IDs] NLM/ PMC4125679
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66. Coşkun H, Bostanci O, Dilege ME, Mihmanli M, Yilmaz B, Akgün I, Yildirim S: Carcinoid tumors of appendix: treatment and outcome. Ulus Travma Acil Cerrahi Derg; 2006 Apr;12(2):150-4
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  • [Title] Carcinoid tumors of appendix: treatment and outcome.
  • BACKGROUND: The aim of this study is to evaluate the clinical and histopathological features and the treatment of carcinoid tumors of the appendix.
  • METHODS: A retrospective review of medical records and pathology specimens of patients with carcinoid tumor of the appendix has been done.
  • RESULTS: The histopathological examination of the appendices revealed carcinoid tumor in 11 out of 6777 (0.16%) patients operated for acute appendicitis.
  • The tumor was localized in the distal 1/3 region in 10 patients and in the proximal 1/3 region in 1 patient.
  • The mean tumor diameter was 0.73+/-0.36 cm (0.3-1.5).
  • Ten patients had classical type carcinoid tumor whereas goblet cell carcinoid tumor was only seen in one patient.
  • CONCLUSION: In tumors with a diameter of 1 to 2 cm, appendectomy is the treatment of choice.
  • No recurrence was detected with tumors smaller than 2 cm with simple appendectomy.
  • [MeSH-major] Appendiceal Neoplasms / epidemiology. Appendiceal Neoplasms / surgery. Carcinoid Tumor / epidemiology. Carcinoid Tumor / surgery

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  • (PMID = 16676255.001).
  • [ISSN] 1306-696X
  • [Journal-full-title] Ulusal travma ve acil cerrahi dergisi = Turkish journal of trauma & emergency surgery : TJTES
  • [ISO-abbreviation] Ulus Travma Acil Cerrahi Derg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Turkey
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67. Safioleas MC, Moulakakis KG, Kontzoglou K, Stamoulis J, Nikou GC, Toubanakis C, Lygidakis NJ: Carcinoid tumors of the appendix. Prognostic factors and evaluation of indications for right hemicolectomy. Hepatogastroenterology; 2005 Jan-Feb;52(61):123-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Carcinoid tumors of the appendix. Prognostic factors and evaluation of indications for right hemicolectomy.
  • BACKGROUND/AIMS: Carcinoid tumors of the appendix are thought to be the most common type of appendiceal neoplasms.
  • Although the vast majority of appendiceal carcinoids behave in a benign fashion, they are considered malignant because they all have the potential for invasion, metastasis and production of physiologically active substances.
  • Also, patients with tumor size more than 1cm underwent a Somatostatin Receptor Scintigraphy (Octreoscan).
  • RESULTS: The most common site for the tumors was the tip of the appendix (18/22).
  • In the rest of the cases, the neoplasms were located in the base (4/22) and in the body (2/22), while in one patient the mesoappendix was invaded.
  • In sixteen patients the tumor size was less than 1cm, in seven patients the tumor diameter was measured to be 1 to 2cm and in one patient the tumor was 3cm.
  • Most of our patients (16/22) underwent only an appendicectomy, while in the rest of them (in the patients with tumor size between 1-2cm and in the patient with invasion of mesoappendix) a right hemicolectomy was performed.
  • No patient was found to have metastatic disease during the operation, while the patient with invasion of the mesoappendix developed metastases in the lung, two years after the operation.
  • CONCLUSIONS: Carcinoid tumors of the appendix, in most cases, are found incidentally during appendicectomies, especially in young females and usually are less than 1cm in size, which is probably the reason of the absence of metastases in all cases.
  • Histological examination and size of the tumor are important factors that contribute to the selection of the surgical treatment and both must be estimated by the surgeons to make the final choice.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Appendiceal Neoplasms / surgery. Carcinoid Tumor / pathology. Carcinoid Tumor / surgery

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  • (PMID = 15783011.001).
  • [ISSN] 0172-6390
  • [Journal-full-title] Hepato-gastroenterology
  • [ISO-abbreviation] Hepatogastroenterology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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68. McGory ML, Maggard MA, Kang H, O'Connell JB, Ko CY: Malignancies of the appendix: beyond case series reports. Dis Colon Rectum; 2005 Dec;48(12):2264-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignancies of the appendix: beyond case series reports.
  • PURPOSE: A comprehensive analysis was performed for five histologic types of appendiceal tumors to compare incidence, clinicopathologic features, survival, and appropriateness of surgery.
  • METHODS: All patients diagnosed with mucinous adenocarcinoma (n = 951), adenocarcinoma (n = 646), carcinoid (n = 435), goblet (n = 369), and signet-ring cell (n = 113) in the Surveillance, Epidemiology, and End Results database (1973-2001) were analyzed.
  • The appropriateness of the operative procedure (i.e. , appendectomy vs. colectomy) was examined by tumor type and size.
  • RESULTS: Tumor incidence, patient demographics, survival outcomes, and appropriateness of surgery varied significantly among the different appendiceal tumor histologies.
  • The most common appendiceal tumors were mucinous.
  • With regard to patient demographics, carcinoids presented at an earlier mean age of 41 years and 71 percent were female (P < 0.001 for both).
  • Overall five-year survival was highest for carcinoid (83 percent) and lowest for signet ring (18 percent).
  • Although current guidelines specify that a right hemicolectomy (rather than an appendectomy) be performed for all noncarcinoid tumors and carcinoid tumors >2 cm, we found that 30 percent of noncarcinoids underwent appendectomy.
  • Similarly, 28 percent of carcinoids >2 cm under-went appendectomy, which is a lesser resection than is indicated.
  • CONCLUSIONS: This study provides a population-based analysis of epidemiology, tumor characteristics, survival, and quality of care for appendiceal carcinomas.
  • This characterization provides a novel description of the presentation and outcomes for malignancies of the appendix and highlights that a substantial number of patients with appendiceal tumors may not be receiving appropriate surgical resection.
  • [MeSH-major] Adenocarcinoma, Mucinous / pathology. Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Carcinoma, Signet Ring Cell / pathology

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  • (PMID = 16258711.001).
  • [ISSN] 0012-3706
  • [Journal-full-title] Diseases of the colon and rectum
  • [ISO-abbreviation] Dis. Colon Rectum
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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69. Cernaianu G, Tannapfel A, Nounla J, Gonzalez-Vasquez R, Wiesel T, Tröbs RB: Appendiceal carcinoid tumor with lymph node metastasis in a child: case report and review of the literature. J Pediatr Surg; 2010 Nov;45(11):e1-5
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  • [Title] Appendiceal carcinoid tumor with lymph node metastasis in a child: case report and review of the literature.
  • Most appendiceal carcinoids (ACs) in children present without lymph node metastasis.
  • Lymph node metastasis is rarely present when primary tumor diameter exceeds 1 cm.
  • We present the extraordinary case of an AC with a primary tumor diameter of 0.7 cm and infiltration of the mesentery, as well as 1 positive lymph node of the mesentery in a 14-year-old boy.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / secondary

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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
  • (PMID = 21034920.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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70. Palanivelu C, Rangarajan M, Annapoorni S, Senthilkumar R, Anand NV: Laparoscopic right hemicolectomy for goblet-cell carcinoid of the appendix: report of a rare case and literature survey. J Laparoendosc Adv Surg Tech A; 2008 Jun;18(3):417-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic right hemicolectomy for goblet-cell carcinoid of the appendix: report of a rare case and literature survey.
  • Tumors of the appendix are rare entities, and the majority of them are discovered accidentally during an investigation for other conditions.
  • Laparoscopic surgery for appendiceal goblet-cell carcinoid (GCC) has only been reported once before.
  • Our patient was incidentally discovered to have an appendiceal tumor and was referred to us for laparoscopy.
  • The tumor involved the body of the appendix and was adherent to the cecum.
  • Histopathology confirmed an appendiceal goblet-cell carcinoid.
  • GCC is a rare tumor of the appendix.
  • Hemicolectomy is indicated in specific situations, such as local involvement or tumor size >2 cm.
  • In our patient, the tumor was adherent to the cecum and tumor size was 5 cm.
  • Laparoscopic surgery for appendiceal tumors is safe, feasible, and even may be beneficial.
  • [MeSH-major] Carcinoid Tumor / surgery. Colectomy
  • [MeSH-minor] Aged. Appendiceal Neoplasms / pathology. Appendiceal Neoplasms / surgery. Humans. Laparoscopy. Male

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  • (PMID = 18503377.001).
  • [ISSN] 1092-6429
  • [Journal-full-title] Journal of laparoendoscopic & advanced surgical techniques. Part A
  • [ISO-abbreviation] J Laparoendosc Adv Surg Tech A
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 18
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71. San Vicente B, Bardají C, Rigol S, Obiols P, Melo M, Bella R: [Retrospective evaluation of carcinoid tumors of the appendix in children]. Cir Pediatr; 2009 Apr;22(2):97-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Retrospective evaluation of carcinoid tumors of the appendix in children].
  • [Transliterated title] Estudio retrospectivo del tumor carcinoide apendicular en niños.
  • Carcinoids of the appendix are rare in children.
  • To investigate the significance of the diagnosis of appendiceal carcinoid tumors in children, we conducted a retrospective study in our institution.
  • In four patients the diagnosis was appendiceal carcinoid.
  • The median tumor diameter was lower than 1 cm and the appropriate treatment was appendectomy.
  • [MeSH-major] Appendix. Carcinoid Tumor. Cecal Neoplasms

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  • (PMID = 19715134.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 14
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72. Louthan O: [Goblet cell carcinoid of the appendix]. Vnitr Lek; 2009 Nov;55(11):1056-9
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  • [Title] [Goblet cell carcinoid of the appendix].
  • [Transliterated title] Karcinoid appendixu s pohárkovými bunkami.
  • Appendiceal goblet cell carcinoids are mixed tumors including neuroendocrine cells and intestinal type of goblet cells.
  • Compared to typical carcinoids, goblet cell carcinoids are malignant tumors with degree of malignity differing from case to case.
  • Carcinoid syndrom is not present.
  • There is poor prognosis in larger tumors.
  • Appendectomy is a sufficient therapeutical approach in small tumors, hemicolectomy should be performed in larger ones.
  • There is limited experience with chemotherapy in metastasizing tumors and sporadic with somatostatin analogues.
  • [MeSH-major] Appendiceal Neoplasms. Carcinoid Tumor

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  • (PMID = 20017437.001).
  • [ISSN] 0042-773X
  • [Journal-full-title] Vnitr̆ní lékar̆ství
  • [ISO-abbreviation] Vnitr Lek
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 18
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73. Terada T: Non-invasive Adenocarcinoma of the Vermiform Appendix: Incidence and Report of Four Cases among 512 Appendectomies. Gastroenterology Res; 2009 Aug;2(4):238-241
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Non-invasive Adenocarcinoma of the Vermiform Appendix: Incidence and Report of Four Cases among 512 Appendectomies.
  • : Tumors of the vermiform appendix are relatively rare.
  • More than 50% of appendiceal tumors are carcinoid tumors.
  • The author reviewed 512 consecutive pathological specimens of appendectomies in last ten years in our pathology laboratory in search for appendiceal tumors.
  • As the results, 4 cases (incidence: 0.8%) of non-invasive adenocarcinoma were found.
  • No other tumors including carcinoid tumors were recognized.
  • Pathologically, all the 4 tumors were non-invasive adenocarcinomas: 2 cases were flat type adenocarcinoma, 1 case was papillary adenocarcinoma, and 1 case was mucinous adenocarcinoma.
  • The results suggest that incidence of appendiceal adenocarcinoma was 0.8% of all appendectomies, and that non-invasive adenocarcinoma of the appendix shows variable morphologies, and that postoperative clinical outcome of non-invasive appendiceal tumor is good.

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  • (PMID = 27942282.001).
  • [ISSN] 1918-2805
  • [Journal-full-title] Gastroenterology research
  • [ISO-abbreviation] Gastroenterology Res
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Keywords] NOTNLM ; Adenocarcinoma / Appendix / Histopathology / Non-invasive carcinoma / appendicitis
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74. Tang LH, Shia J, Soslow RA, Dhall D, Wong WD, O'Reilly E, Qin J, Paty P, Weiser MR, Guillem J, Temple L, Sobin LH, Klimstra DS: Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix. Am J Surg Pathol; 2008 Oct;32(10):1429-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.
  • Appendiceal tumors exhibiting both neuroendocrine and glandular differentiation are uncommon and have caused difficulty in pathologic classification, prediction of prognosis, and clinical management.
  • Previously, such lesions have been variously designated as adenocarcinoid, goblet cell carcinoid (GCC), and mixed adenocarcinoma carcinoid.
  • In this study, we undertook a retrospective investigation of 63 such cases and classified them as typical GCC (group A) and adenocarcinoma ex GCC on the basis of the histologic features of the tumor at the primary site.
  • The adenocarcinoma ex GCC group was further divided into signet ring cell type (group B) and poorly differentiated adenocarcinoma type (group C).
  • The clinical characteristics and prognosis were compared within these groups and with conventional de novo appendiceal adenocarcinomas.
  • Both groups A and B tumors shared a similar immunoprofile, which included generally focal immunoreactivity for neuroendocrine markers, and a normal intestinal type mucin glycoprotein profile (negative MUC1 expression and preserved MUC2 immunoreactivity).
  • The proliferative index was relatively low in these tumors and slightly increased from groups A to B tumors (11% to 16%).
  • Both beta-catenin and E-cadherin exhibited a normal membranous staining pattern in groups A and B tumors.
  • The poorly differentiated adenocarcinomas ex GCC (group C) demonstrated abnormal p53 and beta-catenin immunoreactivity.
  • The mean follow-up time was 49+/-5 (SE) months.
  • The overall disease-specific survival for all subtypes was 77%, with 46% of patients without evidence of disease and 31% alive with disease.
  • All the patients with clinical stage of I or IIA disease had a favorable outcome after appropriate surgery with or without chemotherapy.
  • Although most patients (63%) with GCC presented at an advanced clinical stage, their clinical outcome could be differentiated by subclassification of tumors.
  • The stage IV-matched 5-year survival was 100%, 38%, and 0% for groups A, B, and C, respectively.
  • In conclusion, GCC is a distinctive appendiceal neoplasm that exhibits unique pathologic features and clinical behavior.
  • They display a spectrum of histologic features and possess the potential to transform to an adenocarcinoma phenotype of either signet ring cell or poorly differentiated adenocarcinoma types.
  • Careful evaluation of the morphologic features of GCCs and appropriate pathologic classification are crucial for clinical management and prediction of outcome.
  • Surgical management with right hemicolectomy is recommended after appendectomy for most cases, particularly those with an adenocarcinoma component (groups B and C).
  • [MeSH-major] Adenocarcinoma / pathology. Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Carcinoma, Signet Ring Cell / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Appendectomy. Cell Differentiation. Cell Proliferation. Chemotherapy, Adjuvant. Colectomy. Disease-Free Survival. Female. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Neoplasm Staging. Observer Variation. Retrospective Studies. Terminology as Topic. Time Factors. Treatment Outcome


75. Ng SC, Noursadeghi M, von Herbay A, Vaizey C, Pitcher MC, Flanagan KL: Cytomegalovirus ileitis associated with goblet cell carcinoid tumour of the appendix. J Infect; 2007 Mar;54(3):e153-6
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  • [Title] Cytomegalovirus ileitis associated with goblet cell carcinoid tumour of the appendix.
  • We report a female patient with cytomegalovirus (CMV) terminal ileitis and CMV viraemia, associated with a metastatic goblet cell carcinoid (GCC) tumour of the appendix.
  • [MeSH-major] Appendiceal Neoplasms / complications. Carcinoid Tumor / complications. Cytomegalovirus / isolation & purification. Cytomegalovirus Infections / complications. Ileitis / complications. Ileitis / virology

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  • (PMID = 17049463.001).
  • [ISSN] 1532-2742
  • [Journal-full-title] The Journal of infection
  • [ISO-abbreviation] J. Infect.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antiviral Agents; P9G3CKZ4P5 / Ganciclovir
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76. Ma KW, Chia NH, Yeung HW, Cheung MT: If not appendicitis, then what else can it be? A retrospective review of 1492 appendectomies. Hong Kong Med J; 2010 Feb;16(1):12-7
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  • OBJECTIVE: To audit the appendectomies at our institute, and summarise atypical pathological results with a discussion of appropriate management.
  • Patient demographics, pathological findings, and surgical outcomes were analysed.
  • RESULTS. The overall negative appendectomy rate was 18.2%.
  • Female patients of reproductive age (11-50 years) conferred an independent risk for a higher negative appendectomy rate than other females (28.7% vs 11.5%; P<0.001).
  • Only 4.0% of the appendectomy specimens contained other appendiceal pathologies.
  • Appendiceal diverticulitis was the most common inflammatory pathology, contributing to 2.7% of all appendectomies, followed by granulomatous appendicitis.
  • In this series there were eight carcinoid tumours, three adenocarcinomas, two mucinous cystadenomas; tubular adenoma, metastatic deposition, mucinous cystadenocarcinoma and pseudomyxoma peritonei each occurred in one patient only.
  • Long-term follow-up should be offered to patients with granulomatous appendicitis and neoplastic appendiceal diseases.

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  • [CommentIn] Hong Kong Med J. 2010 Apr;16(2):158 [20354257.001]
  • (PMID = 20124568.001).
  • [ISSN] 1024-2708
  • [Journal-full-title] Hong Kong medical journal = Xianggang yi xue za zhi
  • [ISO-abbreviation] Hong Kong Med J
  • [Language] ENG
  • [Publication-type] Journal Article; Review
  • [Publication-country] China
  • [Number-of-references] 27
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77. Goere D, Elias D: [Appendiceal tumors found at appendectomy]. J Chir (Paris); 2009 Oct;146 Spec No 1:36-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Appendiceal tumors found at appendectomy].
  • [Transliterated title] Diagnostic de tumeur appendiculaire lors d'une appendicectomie.
  • There are three main histologic types of appendiceal tumor: adenoma, adenocarcinoma, and neuroendocrine tumor.
  • Neuroendocrine tumors (carcinoids) are by far the most common and account for two-third of all appendiceal tumors.
  • Rupture of any mucinous tumor-whether spontaneous or occurring during surgery-may result in pseudomyxoma peritonei; treatment of this condition requires complete resection of all lesions followed by hyperthermic intraperitoneal chemotherapy.
  • For unruptured appendiceal tumor, the appendix should be removed by a carcinologic right hemicolectomy if the tumor appears aggressive.
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / surgery
  • [MeSH-minor] Adenocarcinoma / diagnosis. Adenocarcinoma / surgery. Adenoma / diagnosis. Adenoma / surgery. Humans. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / surgery. Peritoneal Neoplasms / prevention & control. Pseudomyxoma Peritonei / prevention & control. Rupture / prevention & control

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  • (PMID = 19846099.001).
  • [ISSN] 0021-7697
  • [Journal-full-title] Journal de chirurgie
  • [ISO-abbreviation] J Chir (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 7
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78. Suzuki O, Ono K, Sekishita Y, Fujimori M, Shiono T, Kondo S: Laparoscopic two-stage surgery for goblet cell carcinoid of the appendix: report of a case and review of the Japanese literature. Surg Laparosc Endosc Percutan Tech; 2006 Apr;16(2):106-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic two-stage surgery for goblet cell carcinoid of the appendix: report of a case and review of the Japanese literature.
  • Goblet cell carcinoid of the appendix is a rare clinical entity exhibiting features of both carcinoid and adenocarcinoma.
  • Here, we present the first report of laparoscopic 2-stage surgery for goblet cell carcinoid with a review of the Japanese literature.
  • A pathologic diagnosis of goblet cell carcinoid, accompanied by the aggressive proliferation, with acute appendicitis was made.
  • Subsequent laparoscopic ileocecal resection was performed, and it was verified that there were neither residual tumor nor lymph node metastases.
  • Because goblet cell carcinoid may be difficult to clinically distinguish from acute appendicitis, pathologic examination is essential.
  • Depending upon the grade of tumor proliferation, additional resection should be considered, and our experience with this case suggests that laparoscopic 2-stage surgery is feasible for the adequate treatment of goblet cell carcinoid without complications.
  • [MeSH-major] Appendectomy / methods. Appendiceal Neoplasms / surgery. Carcinoid Tumor / surgery. Colectomy / methods. Laparoscopy / methods

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  • (PMID = 16773013.001).
  • [ISSN] 1530-4515
  • [Journal-full-title] Surgical laparoscopy, endoscopy & percutaneous techniques
  • [ISO-abbreviation] Surg Laparosc Endosc Percutan Tech
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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79. Fujiyoshi Y, Kuhara H, Eimoto T: Composite glandular-endocrine cell carcinoma of the stomach. Report of two cases with goblet cell carcinoid component. Pathol Res Pract; 2005;200(11-12):823-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Composite glandular-endocrine cell carcinoma of the stomach. Report of two cases with goblet cell carcinoid component.
  • Composite glandular-endocrine cell carcinoma (CGECC) is recognized as a special type of gastric tumor composed of ordinary adenocarcinoma and neuroendocrine tumors.
  • Goblet cell carcinoid (GCC) is a well-established type of appendiceal carcinoid, but the GCC component has not been well delineated in CGECC of the stomach.
  • However, the resected tumors consisted of three components: signet-ring cell carcinoma, GCC, and glandular adenocarcinoma.
  • Although some signet-ring carcinoma cells and goblet carcinoid cells were indistinguishable by hematoxylin and eosin staining, E-cadherin immunostaining disclosed a definitive difference regarding the staining pattern in these cells.
  • Both patients are well, with no recurrent tumor for about 10 years of follow-up.
  • [MeSH-major] Carcinoid Tumor / pathology. Carcinoma, Signet Ring Cell / pathology. Stomach Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Biomarkers, Tumor / metabolism. Cadherins / metabolism. Female. Humans. Immunohistochemistry. Male. Neoplasms, Second Primary. Treatment Outcome


80. Gilboa Y, Fridman E, Ofir K, Achiron R: Carcinoid tumor of the appendix: ultrasound findings in early pregnancy. Ultrasound Obstet Gynecol; 2008 May;31(5):576-8
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  • [Title] Carcinoid tumor of the appendix: ultrasound findings in early pregnancy.
  • Ultrasound examination of a woman in early pregnancy with right lower quadrant abdominal pain demonstrated an edematous appendix with amorphous fluid surrounding the appendix.
  • At laparotomy, these findings were confirmed.
  • On pathological evaluation following surgical removal of the appendix a rare case of carcinoid tumor of the appendix was diagnosed.
  • This is the first description of the transvaginal ultrasound features of a carcinoid tumor of the appendix.
  • [MeSH-major] Appendiceal Neoplasms / ultrasonography. Carcinoid Tumor / ultrasonography. Pregnancy Complications, Neoplastic / ultrasonography

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  • [Copyright] Copyright (c) 2008 ISUOG
  • (PMID = 18393270.001).
  • [ISSN] 1469-0705
  • [Journal-full-title] Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
  • [ISO-abbreviation] Ultrasound Obstet Gynecol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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81. Landry CS, Woodall C, Scoggins CR, McMasters KM, Martin RC 2nd: Analysis of 900 appendiceal carcinoid tumors for a proposed predictive staging system. Arch Surg; 2008 Jul;143(7):664-70; discussion 670
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  • [Title] Analysis of 900 appendiceal carcinoid tumors for a proposed predictive staging system.
  • BACKGROUND: Appendiceal carcinoid tumors (ACTs) are rare, and little is known about the long-term prognosis for these tumors because no staging system exists.
  • DESIGN: Retrospective review of 15 983 patients with carcinoid tumors in the Surveillance Epidemiology and End Results (SEER) database from January 1, 1977, to December 31, 2004.
  • PARTICIPANTS: Nine hundred patients with ACTs (552 females and 348 males; mean age, 47.1 years [age range, 9-89 years]; mean size of the primary tumor, 2.4 cm [range, 0.1-11.5 cm]).
  • RESULTS: Lymph node metastasis was found in 137 patients (24%), and distant metastatic disease in 89 patients (10%).
  • At multivariate analysis, patient age, primary tumor size, histologic features, lymph node involvement, and distant metastasis were significant factors predicting survival.
  • A TNM staging system for ACTs will be helpful not only for physician education about factors that affect the outcome with this disease but also to observe trends in prognosis.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Neoplasm Staging

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  • (PMID = 18645109.001).
  • [ISSN] 1538-3644
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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82. Kidd M, Modlin IM, Mane SM, Camp RL, Shapiro MD: Q RT-PCR detection of chromogranin A: a new standard in the identification of neuroendocrine tumor disease. Ann Surg; 2006 Feb;243(2):273-80
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  • [Title] Q RT-PCR detection of chromogranin A: a new standard in the identification of neuroendocrine tumor disease.
  • OBJECTIVE: Message and protein expression of CgA was examined to evaluate the sensitivity of a PCR-based approach in the detection of covert neuroendocrine (NE) tissue.
  • SUMMARY BACKGROUND DATA: Immunohistochemical (IHC) measurement of chromogranin A (CgA) discriminates gastrointestinal (GI) carcinoids from epithelial tumors.
  • IHC is, however, an insensitive technique to identify micrometastases or delineate subpopulations of NE cells.
  • METHODS: CgA gene expression was examined by Q-RT PCR in GI carcinoids (small intestinal and metastases, n=17, gastric, n=5), appendiceal tumors (n=10), and adenocarcinomas (gastric, n=5, colorectal, n=6).
  • CgA protein expression levels were quantitatively analyzed following IHC by automated quantitative analysis (AQUA) in 2 tissue microarrays (GI carcinoid and GI adenocarcinoma).
  • RESULTS: CgA gene was overexpressed (P<0.001) in GI carcinoids compared with GI adenocarcinomas and normal mucosa.
  • Elevated levels (P<0.00001) were also identified in carcinoid liver and lymph node (LN) metastases.
  • CgA levels were higher (approximately 2-4-fold) in NE appendiceal carcinoids than in adenocarcinoids, but in GI adenocarcinomas were identical to normal mucosa.
  • CgA protein levels were highest in primary GI carcinoids and in liver metastases and significantly elevated (P<0.005) compared with nonmetastatic lesions.
  • CONCLUSIONS: Overexpression of CgA mRNA and protein in GI carcinoids can identify metastatic cells; thus, PCR for CgA can be used to identify micrometastases not evident by light microscopy or IHC as well as define tumors of ambivalent morphologic phenotype.

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  • (PMID = 16432362.001).
  • [ISSN] 0003-4932
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA097050; United States / NCI NIH HHS / CA / R01-CA-097050
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CHGA protein, human; 0 / Chromogranin A; 0 / Chromogranins; 0 / RNA, Neoplasm
  • [Other-IDs] NLM/ PMC1448909
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83. Barry M, Collins CG, McCawley N, McGuinness J, Leahy AL: Synchronous appendiceal tumours. Surgeon; 2007 Apr;5(2):111-3
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  • [Title] Synchronous appendiceal tumours.
  • Primary appendiceal neoplasms are a rare clinical and pathological entity.
  • We report a case of synchronous appendiceal tumours of different histological types which presented as a symptomatic palpable and radiologically apparent mass in the right iliac fossa.
  • This case demonstrates the importance of pre-operative diagnosis of these neoplasms, as it may alter the surgical approach and obviate the need for additional surgery.
  • Furthermore, some of the controversies associated with the management of an appendix mass in the elderly population are discussed.
  • [MeSH-major] Adenocarcinoma, Mucinous / pathology. Appendiceal Neoplasms / pathology. Carcinoid Tumor / pathology. Neoplasms, Multiple Primary / pathology

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  • (PMID = 17450695.001).
  • [ISSN] 1479-666X
  • [Journal-full-title] The surgeon : journal of the Royal Colleges of Surgeons of Edinburgh and Ireland
  • [ISO-abbreviation] Surgeon
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
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84. Basic-Jukic N, Furic-Cunko V, Coric M, Bubic-Filipi LJ, Kastelan Z, Pasini J, Kes P: Appendiceal carcinoid and mucinous cystadenoma in renal transplant recipients: case reports. Transplant Proc; 2010 Jun;42(5):1704-7
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  • [Title] Appendiceal carcinoid and mucinous cystadenoma in renal transplant recipients: case reports.
  • There is an increased incidence of tumors among renal transplant patients, which are associated with immunosuppression.
  • Carcinoids are rare neuroendocrine tumors that arise from the enterochromaffin cells.
  • Although appendiceal carcinoid tumors are the commonest malignant neoplasms affecting the appendix, and mucinous cystadenoma is the commonest benign appendiceal neoplasm, they have not been reported in immunosuppressed patients.
  • We present two renal transplant recipients who developed combined appendiceal carcinoid and mucinous cystadenoma.
  • [MeSH-major] Appendiceal Neoplasms / etiology. Cystadenoma, Mucinous / etiology. Kidney Transplantation / adverse effects
  • [MeSH-minor] Adult. Female. Humans. Kidney Failure, Chronic / surgery. Magnetic Resonance Imaging. Male. Neoplasms / epidemiology. Neoplasms / etiology. Neoplasms / pathology. Neoplasms / surgery. Ovarian Neoplasms / pathology. Ovarian Neoplasms / surgery

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  • (PMID = 20620505.001).
  • [ISSN] 1873-2623
  • [Journal-full-title] Transplantation proceedings
  • [ISO-abbreviation] Transplant. Proc.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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85. Mukensnabl P, Michal M, Hadravská S: [Carcinoids of the appendix]. Rozhl Chir; 2005 Apr;84(4):197-200
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Carcinoids of the appendix].
  • The appendical carcinoids represent a heterogeneous group of tumors of different morphology, biological features and treatment.
  • Our study aims to inform clinical practitioners, mainly surgeons and oncologists, about individual types of the appendical carcinoids, which are considered the most frequent carcinoid tumors the clinical practitioners come into contact with at all levels of the hospital care.
  • Our work describes the types of the appendical tumors from the histological point of view, as well as from the prognostic and therapeutical points of view.
  • At the end of the study report, a brief table summarizing the most important features of the individual histological types of the appendical carcinoids, is attached.
  • [MeSH-major] Carcinoid Tumor
  • [MeSH-minor] Appendiceal Neoplasms / diagnosis. Appendiceal Neoplasms / pathology. Appendiceal Neoplasms / surgery

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  • (PMID = 15984148.001).
  • [ISSN] 0035-9351
  • [Journal-full-title] Rozhledy v chirurgii : měsíčník Československé chirurgické společnosti
  • [ISO-abbreviation] Rozhl Chir
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 8
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86. Tang LH: Epithelial neoplasms of the appendix. Arch Pathol Lab Med; 2010 Nov;134(11):1612-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Epithelial neoplasms of the appendix.
  • CONTEXT: The appendix gives rise to an array of epithelial neoplasms showing glandular or neuroendocrine differentiation, and some tumors with elements of both cell types.
  • Although some appendiceal neoplasms resemble their counterparts in the small and large intestines (conventional adenocarcinoma and carcinoid tumor), the appendix also gives rise to relatively unique entities including mucinous neoplasms and goblet cell carcinoid tumors, which present a challenge in pathologic classification and clinical management.
  • OBJECTIVE: To review clinical and diagnostic issues for 3 pathologic types of epithelial neoplasms of the appendix:.
  • (1) adenocarcinoma, with specific focus on mucinous neoplasm;.
  • (2) goblet cell carcinoid tumor and associated adenocarcinoma; and (3) typical carcinoid tumor.
  • CONCLUSIONS: The most important issue in pathologic assessment of epithelial tumors of the appendix is to understand the clinical implications inherent in the diagnosis.
  • [MeSH-major] Adenocarcinoma / pathology. Appendiceal Neoplasms / pathology. Appendix / pathology. Carcinoid Tumor / pathology

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  • (PMID = 21043814.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
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87. Graham RP, Williams NP, West KA: Primary epithelial tumours of the appendix in a black population: a review of cases. World J Gastroenterol; 2009 Mar 28;15(12):1472-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary epithelial tumours of the appendix in a black population: a review of cases.
  • AIM: To determine the prevalence, histologic types and clinical features of primary epithelial tumours of the vermiform appendix in a predominantly black population.
  • METHODS: All cases of primary tumours of the appendix identified by review of the histopathology records at the University of the West Indies between January 1987 and June 2007 were selected.
  • Non-epithelial tumours were excluded.
  • RESULTS: Forty-two primary epithelial tumours were identified out of 6,824 appendectomies yielding a prevalence rate of approximately 0.62%.
  • Well-differentiated neuroendocrine cell tumours (carcinoids, 47.6%) and benign non-endocrine cell tumours (adenomas, 45.2%) were most common with nearly equal frequency.
  • Carcinoid tumours occurred in younger patients (mean age 32 years), with a male-to-female ratio of 1.2:1.
  • CONCLUSION: Appendiceal epithelial tumours are rare in our experience, and are represented principally by carcinoid tumours and adenomas.
  • Carcinoid tumours occurred in younger patients but were slightly more common in men than women.
  • Tumours were not suspected clinically and were diagnosed incidentally in specimens submitted for acute appendicitis supporting the need for histological evaluation in all resection specimens.
  • [MeSH-major] African Continental Ancestry Group / statistics & numerical data. Appendiceal Neoplasms / epidemiology. Appendiceal Neoplasms / pathology
  • [MeSH-minor] Adenocarcinoma / epidemiology. Adenocarcinoma / pathology. Adenoma / epidemiology. Adenoma / pathology. Adolescent. Adult. Aged. Aged, 80 and over. Carcinoid Tumor / epidemiology. Carcinoid Tumor / pathology. Female. Humans. Male. Middle Aged. Prevalence. Retrospective Studies. West Indies / epidemiology. Young Adult

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  • (PMID = 19322920.001).
  • [ISSN] 2219-2840
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC2665141
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88. Zhang HY, Rumilla KM, Jin L, Nakamura N, Stilling GA, Ruebel KH, Hobday TJ, Erlichman C, Erickson LA, Lloyd RV: Association of DNA methylation and epigenetic inactivation of RASSF1A and beta-catenin with metastasis in small bowel carcinoid tumors. Endocrine; 2006 Dec;30(3):299-306
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  • [Title] Association of DNA methylation and epigenetic inactivation of RASSF1A and beta-catenin with metastasis in small bowel carcinoid tumors.
  • We analyzed promoter methylation of RASSF1A, CTNNB1, CDH1, LAMB3, LAMC2, RUNX3, NORE1A, and CAV1 using methylation-specific PCR in 33 cases of small bowel carcinoid with both matched primary and metastatic tumors.
  • The methylation status of RASSF1A and CTNNB1 were also determined in six primary appendiceal carcinoid tumors.
  • Immunohistochemical analyses for RASSF1A and beta-catenin were performed in 28 matched primary and metastatic tumors.
  • RASSF1A and CTNNB1 were unmethylated in appendiceal carcinoids.
  • Methylation of RASSF1A and CTNNB1 promoters was more frequent in metastatic compared to primary tumors (p = 0.013 and 0.004, respectively).
  • Western blot results for RASSF1A and beta-catenin supported the methylation-specific PCR findings.
  • These results suggest that increased methylation of RASSF1A and CTNNB1 may play important roles in progression and metastasis of small bowel carcinoid tumors.
  • [MeSH-major] Carcinoid Tumor / pathology. DNA Methylation. Intestinal Neoplasms / pathology. Tumor Suppressor Proteins / genetics. beta Catenin / genetics
  • [MeSH-minor] Azacitidine / analogs & derivatives. Blotting, Western. Cell Line, Tumor. CpG Islands. DNA Mutational Analysis. Enterochromaffin Cells / metabolism. Epigenesis, Genetic. Humans. Immunohistochemistry. Neoplasm Metastasis / genetics. Promoter Regions, Genetic

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  • (PMID = 17526942.001).
  • [ISSN] 1355-008X
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins; 0 / beta Catenin; 776B62CQ27 / decitabine; M801H13NRU / Azacitidine
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89. Alsaad KO, Serra S, Chetty R: Combined goblet cell carcinoid and mucinous cystadenoma of the vermiform appendix. World J Gastroenterol; 2009 Jul 21;15(27):3431-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Combined goblet cell carcinoid and mucinous cystadenoma of the vermiform appendix.
  • Goblet cell carcinoid is an uncommon primary tumor of the vermiform appendix, characterized by dual endocrine and glandular differentiation.
  • Whether goblet cell carcinoid represents a morphological variant of appendiceal classical carcinoid or a mucin-producing adenocarcinoma is a matter of conjecture.
  • Rare cases of goblet cell carcinoid with other concomitant appendiceal epithelial neoplasms have been documented.
  • In this report, we describe a rare case of combined appendiceal goblet cell carcinoid and mucinous cystadenoma, and discuss the possible histopathogenesis of this combination.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Appendix / pathology. Carcinoid Tumor / pathology. Cystadenoma, Mucinous / pathology
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Female. Humans. Middle Aged

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  • (PMID = 19610147.001).
  • [ISSN] 2219-2840
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Other-IDs] NLM/ PMC2712907
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90. Ellis L, Shale MJ, Coleman MP: Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971. Am J Gastroenterol; 2010 Dec;105(12):2563-9
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  • [Title] Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971.
  • OBJECTIVES: The epidemiology of gastrointestinal neuroendocrine tumors (GI-NETs) is poorly understood.
  • Recent analyses have suggested changes in the incidence and distribution of such tumors, but have generally used data sets containing small patient numbers.
  • RESULTS: In all, 10,324 cases of GI-NETs were identified.
  • The anatomic distribution of tumors in the latest period analyzed was stomach 12%, small intestine 29%, appendix 38%, colon 13%, and rectum 8%.
  • The largest absolute increase in incidence was seen in the appendix (from 0.03 to 0.41 in men; from 0.05 to 0.59 in women).
  • Sex-specific incidence rates for gastric, colonic, and rectal NETs are similar, whereas appendiceal lesions were more common in females, and small intestinal tumors in men.
  • Such changes may partly reflect changes in classification or improved detection through the increased use of endoscopy and imaging techniques.
  • In view of the magnitude of these changes, particularly for gastric tumors, further studies to examine the underlying etiology of these changes are urgently indicated.
  • [MeSH-major] Carcinoid Tumor / epidemiology. Gastrointestinal Neoplasms / epidemiology. Neuroendocrine Tumors / epidemiology

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  • (PMID = 20823835.001).
  • [ISSN] 1572-0241
  • [Journal-full-title] The American journal of gastroenterology
  • [ISO-abbreviation] Am. J. Gastroenterol.
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / / 11700; United Kingdom / Cancer Research UK / / C1336 / A5735
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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91. Nezhat C, Datta MS, Defazio A, Nezhat F, Nezhat C: Natural orifice-assisted laparoscopic appendectomy. JSLS; 2009 Jan-Mar;13(1):14-8
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  • METHODS: We reviewed cases of 42 patients who underwent total laparoscopic hysterectomy or laparoscopic-assisted vaginal hysterectomy followed by appendectomy, performed by applying a stapler and removing the appendix transvaginally.
  • By using a small-diameter laparoscope, the appendix was mobilized, especially in patients with adhesions, endometriosis, or retrocecal appendix, to facilitate transvaginal access with the stapler.
  • RESULTS: All procedures were performed successfully without intraoperative or major postoperative complications.
  • Appendiceal pathology included serosal adhesions (14), fibrous obliteration of the lumen (12), endometriosis (4), serositis (2), and carcinoid tumor (1), among others.

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  • [ISSN] 1086-8089
  • [Journal-full-title] JSLS : Journal of the Society of Laparoendoscopic Surgeons
  • [ISO-abbreviation] JSLS
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92. Modlin IM, Kidd M, Latich I, Zikusoka MN, Eick GN, Mane SM, Camp RL: Genetic differentiation of appendiceal tumor malignancy: a guide for the perplexed. Ann Surg; 2006 Jul;244(1):52-60
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  • [Title] Genetic differentiation of appendiceal tumor malignancy: a guide for the perplexed.
  • OBJECTIVE: To use differential gene expression of candidate markers to discriminate benign appendiceal carcinoids (APCs) from malignant and mixed cell APCs.
  • SUMMARY BACKGROUND DATA: Controversy exists in regard to the appropriate surgical management of APCs since it is sometimes difficult to predict tumor behavior using traditional pathologic criteria.
  • METHODS: Total RNA was isolated using TRIzol reagent from 42 appendiceal samples, including appendiceal carcinoids identified at exploration for appendicitis (no evidence of metastasis; n = 16), appendicitis specimens (n = 11), malignant appendiceal tumors (> 1.5 cm, evidence of metastatic invasion; n = 7), and mixed (goblet) cell appendiceal adenocarcinoids (n = 3), normal appendiceal tissue (n = 5), and 5 colorectal cancers.
  • RESULTS: CgA message was elevated (> 1000-fold, P < 0.05) in all tumor types.
  • NAP1L1 was elevated (> 10-fold, P < 0.03) in both malignant and goblet cell adenocarcinoids compared with normal and incidental lesions (P < 0.006).
  • MAGE-D2 and MTA1 message were significantly elevated (> 10-fold, P < 0.01) in the malignant and goblet cell adenocarcinoid tumors but not in the appendicitis-associated carcinoids or normal mucosa.
  • The apoptotic marker, NALP1, was overexpressed (> 50-fold, P < 0.05) in the appendicitis-associated and malignant appendiceal carcinoids but was significantly decreased (> 10-fold, P < 0.05) in the goblet cell adenocarcinoids.
  • Elevated CgA transcript and protein levels indicative of a carcinoid tumor were identified in one acute appendicitis sample with no histologic evidence of a tumor.
  • CONCLUSIONS: These data demonstrate that malignant APCs and goblet cell adenocarcinoids have elevated expression of NAP1L1, MAGE-D2, and MTA1 compared with appendiceal carcinoids identified at surgery for appendicitis.
  • This and the differences in NALP1 gene expression (decreased in goblet cell adenocarcinoids) provide a series of molecular signatures that differentiate carcinoids of the appendix.
  • CgA identified all appendiceal tumors as well as covert lesions, which may be more prevalent than previously recognized.
  • The molecular delineation of malignant appendiceal tumor potential provides a scientific basis to define the appropriate surgical management as opposed to morphologic assessment alone.

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  • (PMID = 16794389.001).
  • [ISSN] 0003-4932
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA097050; United States / NCI NIH HHS / CA / R01-CA-097050
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / Antigens, Neoplasm; 0 / Apoptosis Regulatory Proteins; 0 / Biomarkers, Tumor; 0 / Cell Cycle Proteins; 0 / Chromogranin A; 0 / Chromogranins; 0 / Genetic Markers; 0 / MAGED2 protein, human; 0 / NAP1L1 protein, human; 0 / NLRP1 protein, human; 0 / Nuclear Proteins; 0 / Nucleosome Assembly Protein 1; 0 / Repressor Proteins; EC 3.5.1.- / Mta1 protein, human; EC 3.5.1.98 / Histone Deacetylases
  • [Other-IDs] NLM/ PMC1570599
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93. Alsaad KO, Serra S, Perren A, Hsieh E, Chetty R: CK19 and CD99 immunoexpression profile in goblet cell (mucin-producing neuroendocrine tumors) and classical carcinoids of the vermiform appendix. Int J Surg Pathol; 2007 Jul;15(3):252-7
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  • [Title] CK19 and CD99 immunoexpression profile in goblet cell (mucin-producing neuroendocrine tumors) and classical carcinoids of the vermiform appendix.
  • The immunoexpression of CK19 recently has been identified as a marker of poor prognosis in pancreatic endocrine tumors and hepatocellular carcinoma.
  • The purpose of this study was to explore CK19 and CD99 immunostaining in mucin-producing neuroendocrine (goblet cell) and classical carcinoids of the appendix.
  • Eighteen goblet cell carcinoids (GCCs) and 20 classic carcinoids were stained with CK19, CD99, and Ki-67, and these results were correlated with known pathological features of aggression: extent of invasion, mitoses, necrosis, and histological pattern.
  • All 18 GCCs were CK19 strongly positive, whereas 16/20 classic carcinoids were also CK19 positive.
  • Fourteen of 18 GCCs and 14/20 classic carcinoids were CD99 positive.
  • CK19/CD99 immunoexpression did not correlate with extent of tumor invasion and mesoappendiceal extension, mitotic activity, Ki-67 labeling index, presence of extracellular mucinous pools dissecting muscle, and angiolymphatic and perineural/neural invasion.
  • There is no difference in the immunostaining for CK19 and CD99 between GCCs and classic carcinoids, and both types of neuroendocrine tumor show the same extent of expression of both markers.
  • [MeSH-major] Antigens, CD / metabolism. Appendiceal Neoplasms / metabolism. Carcinoid Tumor / metabolism. Cell Adhesion Molecules / metabolism. Keratin-19 / metabolism
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Cell Proliferation. Gene Expression Regulation, Neoplastic. Humans. Neoplasm Invasiveness / pathology

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  • (PMID = 17652531.001).
  • [ISSN] 1066-8969
  • [Journal-full-title] International journal of surgical pathology
  • [ISO-abbreviation] Int. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Keratin-19
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94. Bolanowski M, Jarzab B, Handkiewicz-Junak D, Jeziorski A, Kos-Kudła B, Zajecki W, oraz Pozostali Uczestnicy Konferencji Okragłego Stołu: [Neuroendocrine tumors of the small intestine and the appendix - management guidelines (recommended by The Polish Network of Neuroendocrine Tumors)]. Endokrynol Pol; 2008 Jan-Feb;59(1):87-96
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  • [Title] [Neuroendocrine tumors of the small intestine and the appendix - management guidelines (recommended by The Polish Network of Neuroendocrine Tumors)].
  • Polish recommendations regarding management of patients suffering from neuroendocrine tumors of small intestine and appendix are presented.
  • Small intestine, especially ileum represent most common origin of these tumors.
  • Majority of them are well differentiated and grow slowly.
  • Rarely, they are less differentiated with fast growth and poor prognosis.
  • In 4-10% of patients typical symptoms of carcinoid syndrome are present.
  • Chromogranin A is useful in the laboratory diagnostics, and urinary excretion of 5-hydroxyindoloacetic acid is helpful for the diagnostics and monitoring of the disease.
  • The treatment of choice in the neuroendocrine tumors of small intestine and appendix is radical or palliative surgery, if possible using endoscopy.
  • The crucial in biotherapy is somatostatin analogs application, possible in symptomatic treatment of hormonally functioning tumors.
  • This is treatment of choice in carcinoid crisis.
  • Interferon alfa could be applied because of the same indications as somatostatin analogs, except for carcinoid crisis.
  • Chemotherapy is less successful in disseminated or locally advanced intestinal neuroendocrine tumors, so radioisotope therapy should be considered in each case of unresectable tumor.
  • [MeSH-major] Intestinal Neoplasms / diagnosis. Intestinal Neoplasms / therapy. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / therapy. Practice Guidelines as Topic
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Appendiceal Neoplasms / diagnosis. Appendiceal Neoplasms / therapy. Clinical Competence. Combined Modality Therapy / methods. Endoscopy, Gastrointestinal / methods. Humans. Intestine, Small. Neoplasm Staging. Physical Examination. Poland. Risk Factors

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  • (PMID = 18335403.001).
  • [ISSN] 0423-104X
  • [Journal-full-title] Endokrynologia Polska
  • [ISO-abbreviation] Endokrynol Pol
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Investigator] Bar-Andziak E; Cwikła J; de Herder W; Dzielicki J; Falconi M; Foltyn W; Gaciong Z; Hubalewska-Dydejczyk A; Kowalska A; Krolicki L; Krzyzanowska-Swiniarska B; Kryszałowicz B; Kvols L; Nasierowska-Guttmejer A; O'Toole D; Kunikowska J; Lampe P; Matyja V; Mełen-Mucha G; Milewicz A; Nowak A; Nowakowska-Duława E; Omyła-Staszewska J; Pajak J; Rudzki S; Rydzewska G; Sowinski J; Starzynska T; Strzelczyk J; Sworczak K; Syrenicz A; Szawlowski A; Tomaszewska RA; Wasko-Czopnik D; Wronski M; Zemczak A; Zgliczynski W
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95. Roy P, Chetty R: Goblet cell carcinoid tumors of the appendix: An overview. World J Gastrointest Oncol; 2010 Jun 15;2(6):251-8
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  • [Title] Goblet cell carcinoid tumors of the appendix: An overview.
  • Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively.
  • Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural.
  • Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted.
  • The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation.
  • The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass.
  • Atypia is usually minimal, but carcinomatous growth patterns may be seen.
  • These may be of signet ring cell type or poorly differentiated adenocarcinoma.
  • Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q).
  • The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix.
  • The most important prognostic factor is the stage of disease.
  • There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.

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  • (PMID = 21160637.001).
  • [ISSN] 1948-5204
  • [Journal-full-title] World journal of gastrointestinal oncology
  • [ISO-abbreviation] World J Gastrointest Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC2998842
  • [Keywords] NOTNLM ; Appendiceal neoplasm / Goblet cell carcinoid / Mucin-producing neuroendocrine tumor of appendix
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96. Orta L, Trindade AJ, Luo J, Harpaz N: Appendiceal mucinous cystadenoma is a neoplastic complication of IBD: case-control study of primary appendiceal neoplasms. Inflamm Bowel Dis; 2009 Mar;15(3):415-21
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  • [Title] Appendiceal mucinous cystadenoma is a neoplastic complication of IBD: case-control study of primary appendiceal neoplasms.
  • BACKGROUND: IBD is a risk factor for development of colorectal neoplasia.
  • Although IBD frequently involves the appendix microscopically, it is uncertain whether it also predisposes to appendiceal neoplasia.
  • METHODS: We performed a retrospective case-control study of incidental appendiceal neoplasms in colectomy specimens of adults with and without IBD (cases and controls, respectively) based on surgical pathology records spanning 54 months.
  • To minimize referral bias, patients were excluded if they had preoperative clinical evidence or a principal pathologic diagnosis of appendiceal disease.
  • RESULTS: Eleven appendiceal cystadenomas and 6 appendiceal carcinoid tumors were identified among 705 IBD cases (377 ulcerative colitis, 317 Crohn's disease, 11 indeterminate colitis) and 498 non-IBD controls meeting our inclusion criteria.
  • There was no significant difference in prevalence of cyst adenomas between the cases and controls (9/705 [1.3%] versus 2/498 [0.4%], respectively, OR 3.2 [95% CI 0.7-14.9]).
  • However, cyst adenomas were 15-fold more prevalent among cases with synchronous colorectal neoplasia compared with controls (4/69 [5.8%] versus 2/498 [0.4%], OR 15.3 [95% CI 2.7-85]) and 8-fold higher compared with cases without synchronous neoplasia (4/69 [5.8%] versus 5/636 [0.8%], OR 7.8 [95% CI 2.0-29.6]).
  • Appendiceal carcinoids were equally prevalent in cases and controls (4/705 [0.6%] versus 2/498 [0.4%], OR 1.4 [95% CI 0.3-7.8]), cases with synchronous neoplasia and controls (1/69 [1.4%] versus 2/498 [0.4%], OR 3.6 [95% CI 0.3-40.8]), and cases with and without synchronous colorectal neoplasia (1/69 [1.4%] versus 3/636 [0.5%], OR 3.1 [95% CI 0.3-30.2]).
  • CONCLUSIONS: IBD with synchronous colorectal dysplasia or cancer is a risk factor for development of appendiceal cystadenomas, implicating this tumor as a neoplastic complication of IBD.
  • IBD does not predispose to the development of appendiceal carcinoids.
  • [MeSH-major] Appendiceal Neoplasms / etiology. Cystadenoma, Mucinous / etiology. Inflammatory Bowel Diseases / complications
  • [MeSH-minor] Adult. Aged. Appendectomy. Colectomy. Colonoscopy. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Staging. Retrospective Studies. Time Factors. Treatment Outcome

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  • [CommentIn] Inflamm Bowel Dis. 2010 May;16(5):735 [19705419.001]
  • (PMID = 18989894.001).
  • [ISSN] 1536-4844
  • [Journal-full-title] Inflammatory bowel diseases
  • [ISO-abbreviation] Inflamm. Bowel Dis.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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97. Gungor T, Altinkaya O, Ozat M, Sirvan L, Yalcin H, Mollamahmutoglu L: Primary adenocarcinoid tumor of the ovary arising in mature cystic teratoma. A case report. Eur J Gynaecol Oncol; 2009;30(1):110-2
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  • [Title] Primary adenocarcinoid tumor of the ovary arising in mature cystic teratoma. A case report.
  • Primary carcinoid tumors of the ovary are rare, forming 0.3 % of all carcinoid tumors and less than 0.1 % of ovarian malignancies.
  • We present a case of a 47-year-old woman with abnormal vaginal bleeding and abdominal discomfort.
  • At laparoscopy, a smooth surfaced, firm and mobile right adnexal mass with solid and cystic portions was detected and the tumor was excised.
  • Pathologic and immunohistochemical examination revealed carcinoid tumor.
  • After the evaluation of gastrointestinal and respiratory systems by imaging and endoscopic studies, no evidence of tumor tissue or metastases were found.
  • Based on this finding, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and appendectomy were performed and pelvic-paraaortic lymph nodes were also removed.
  • All histological findings were similar to the features of mature cystic teratoma and carcinoid tumor.
  • Examination of the resected lymphatic, omental and appendiceal tissue indicated no tumoral invasion.
  • Thus, the diagnosis was primary ovarian carcinoid arising from a mature cystic teratoma.
  • This case, in the setting of a normal appendix and negative workup for an extraovarian origin, is one of a primary ovarian adenocarcinoid tumor.
  • The primary carcinoid tumor should be differentiated from a metastatic carcinoid inside the ovary, which is always bilateral.
  • It is important to be aware of this unusual entity in diagnoses of ovarian tumors, even in the absence of any clinical symptoms of carcinoid syndrome.
  • [MeSH-major] Adenocarcinoma, Mucinous / pathology. Carcinoid Tumor / pathology. Neoplasms, Second Primary. Ovarian Neoplasms / pathology. Teratoma / pathology


98. Korkontzelos I, Papanicolaou S, Tsimoyiannis I, Kitsiou E, Stefos T, Tsanadis G, Antoniou N: Large carcinoid tumor of the appendix during pregnancy. Eur J Obstet Gynecol Reprod Biol; 2005 Feb 1;118(2):255-7
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  • [Title] Large carcinoid tumor of the appendix during pregnancy.
  • Diagnosis of acute appendicitis is considered more difficult in pregnant than in non-pregnant women.
  • The appendicial carcinoid tumor is a lesion that most frequently is discovered incidentally in the removed organ.
  • We report a rare case of an unruptured acute appendicitis during pregnancy, which proved to be a large carcinoid tumor.
  • [MeSH-major] Appendiceal Neoplasms / diagnosis. Carcinoid Tumor / diagnosis. Pregnancy Complications, Neoplastic


99. Tannapfel A, Wittekind C: [The current TNM system for gastrointestinal tumors part II]. Pathologe; 2010 Sep;31(5):348-52
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  • [Title] [The current TNM system for gastrointestinal tumors part II].
  • [Transliterated title] Aktuelles TNM-System der gastrointestinalen Tumoren Teil II.
  • Entirely new classifications are those for gastrointestinal stromal tumours, gastrointestinal neuroendocrine tumours, intrahepatic cholangiocarcinoma and perihilar extrahepatic bile duct carcinomas.
  • Major and praxis-relevant alterations concern colorectal tumours and include new classifications of carcinomas and carcinoids of the appendix.
  • Minor alterations are seen in the classification of hepatocellular carcinomas.
  • No changes were made for tumours of the anal canal, the gallbladder (excluding the inclusion of tumours of the cystic duct) and tumours of the pancreas and the ampulla of Vater.
  • [MeSH-major] Digestive System Neoplasms / pathology. Neoplasm Staging / methods
  • [MeSH-minor] Ampulla of Vater / pathology. Appendiceal Neoplasms / classification. Appendiceal Neoplasms / pathology. Carcinoid Tumor / classification. Carcinoid Tumor / pathology. Carcinoma, Hepatocellular / classification. Carcinoma, Hepatocellular / pathology. Cholangiocarcinoma / classification. Cholangiocarcinoma / pathology. Colorectal Neoplasms / classification. Colorectal Neoplasms / pathology. Common Bile Duct Neoplasms / pathology. Disease Progression. Gallbladder Neoplasms / classification. Gallbladder Neoplasms / pathology. Gastrointestinal Stromal Tumors / classification. Gastrointestinal Stromal Tumors / pathology. Humans. Lymphatic Metastasis / pathology. Mitotic Index. Neoplasm Invasiveness / pathology. Neuroendocrine Tumors / classification. Neuroendocrine Tumors / pathology. Pancreatic Neoplasms / classification. Pancreatic Neoplasms / pathology. Prognosis. Rectal Neoplasms / classification. Rectal Neoplasms / pathology

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  • [CommentIn] Pathologe. 2010 Sep;31(5):353-4 [20809402.001]
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  • (PMID = 20798945.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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100. Fornaro R, Frascio M, Sticchi C, De Salvo L, Stabilini C, Mandolfino F, Ricci B, Gianetta E: Appendectomy or right hemicolectomy in the treatment of appendiceal carcinoid tumors? Tumori; 2007 Nov-Dec;93(6):587-90
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  • [Title] Appendectomy or right hemicolectomy in the treatment of appendiceal carcinoid tumors?
  • AIMS AND BACKGROUND: Carcinoids of the appendix continue to be of interest, despite their low incidence.
  • There is still considerable controversy surrounding these tumors, especially with regard to the role of right hemicolectomy in the surgical management.
  • The clinical data were reviewed for demographic details, tumor size, localization in the appendix, histological patterns and surgical procedures.
  • All patients underwent appendectomy including removal of the mesenteriolum, and in one of them a right hemicolectomy was performed 3 weeks later.
  • RESULTS: Seven patients (0.9% of all appendectomies) were reported to have carcinoid tumors of the appendix.
  • None suffered from the carcinoid syndrome.
  • The site of the tumor was the apex of the appendix in 4 cases, the body in 2 cases and the base in 1 case.
  • Mean tumor diameter was 8 mm (range, 5-29 mm); in 6 patients it was <2 cm.
  • Treatment was appendectomy in all cases; additional right hemicolectomy was necessary in one case because of a tumor of more than 2 cm with invasion of the mesoappendix and lymph nodes.
  • Six patients are without disease, while 1 patient (the one who underwent a right hemicolectomy) developed metastases in the liver 6 years after the operation.
  • CONCLUSIONS: According to current guidelines, an appendectomy may be performed for small carcinoid tumors (<1 cm).
  • Reasons for more extensive surgery than appendectomy are tumor size >2 cm, lymphatic invasion, lymph node involvement, spread to the mesoappendix, tumor-positive resection margins, and cellular pleomorphism with a high mitotic index.
  • Tumor size is still considered the most important prognostic factor, with a presumed increase in the risk of metastasis for tumors greater than 2.0 cm.
  • The accepted treatment of such tumors is a right hemicolectomy.
  • However, there is no evidence demonstrating a survival benefit for right hemicolectomy over simple appendectomy in patients with carcinoids greater than 2.0 cm in diameter.
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / pathology. Appendiceal Neoplasms / surgery. Carcinoid Tumor / secondary. Carcinoid Tumor / surgery. Colectomy
  • [MeSH-minor] Adolescent. Adult. Female. Humans. Liver Neoplasms / secondary. Lymphatic Metastasis. Male. Middle Aged. Prognosis. Survival Analysis. Treatment Outcome

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  • (PMID = 18338494.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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