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1. Roy P, Chetty R: Goblet cell carcinoid tumors of the appendix: An overview. World J Gastrointest Oncol; 2010 Jun 15;2(6):251-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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 histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy.
  • 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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2. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • We have identified 5 differentially expressed genes (a mitosis-regulatory gene NAP1L1, an adhesin MAGE-D2, an estrogen-antagonist, the metastasis marker MTA1, the apoptotic marker NALP, and chromogranin A) that define gut neuroendocrine cell behavior.
  • 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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3. Carrasquillo JA, Chen CC: Molecular imaging of neuroendocrine tumors. Semin Oncol; 2010 Dec;37(6):662-79

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular imaging of neuroendocrine tumors.
  • Neuroendocrine tumors (NET) are a heterogeneous group of tumors that arise from neuroendocrine cells.
  • These tumors may arise from various organs, including lung, thymus, thyroid, stomach, duodenum, small bowel, large bowel, appendix, pancreas, adrenal, and skin.
  • Most are well differentiated and have the ability to produce biogenic amines and various hormones.
  • In fact, the unique secretory characteristics of these tumors lend themselves to imaging by molecular imaging modalities, which can target specific metabolic pathways or receptors.
  • Neuroendocrine cells have a variety of such target receptors and pathways for which radiopharmaceuticals have been developed, including [(123)I/(131)I]-metaiodobenzylguanidine (MIBG), [(111)In]pentetreotide, [(68)Ga] somatostatin analogs, [(18)F] fluorodeoxyglucose (FDG), [(11)C/(18)F] dihydroxyphenylalanine (DOPA), [(11)C] 5-hydroxytryptophan (5-HTP) (99m)Tc pentavalent dimercaptosuccinic acid ([(99m)Tc] (V) DMSA, and [(18)F] fluorodopamine (FDA).
  • [MeSH-major] Molecular Imaging / methods. Neuroendocrine Tumors / diagnostic imaging. Neuroendocrine Tumors / pathology. Radiopharmaceuticals

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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
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  • (PMID = 21167384.001).
  • [ISSN] 1532-8708
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z99 CL999999
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals
  • [Other-IDs] NLM/ NIHMS546111; NLM/ PMC4003904
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4. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Neuroendocrine tumors of the small intestine and the appendix - management guidelines (recommended by The Polish Network of Neuroendocrine Tumors)].
  • [Transliterated title] Guzy neuroendokrynne jelita cienkiego i wyrostka robaczkowego ( zasady postepowania rekomendowane przez Polska Siec Guzow Neuroendokrynnych).
  • 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.
  • Symptoms are atypical, diagnosis could be often accidental.
  • 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.
  • 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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5. Dupre MP, Jadavji I, Matshes E, Urbanski SJ: Diverticular disease of the vermiform appendix: a diagnostic clue to underlying appendiceal neoplasm. Hum Pathol; 2008 Dec;39(12):1823-6
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  • [Title] Diverticular disease of the vermiform appendix: a diagnostic clue to underlying appendiceal neoplasm.
  • Acquired diverticula of the vermiform appendix are rare and arise as a result of different pathogenetic mechanisms.
  • One of the etiologies includes proximally located, often unsuspected small neoplasms.
  • Although the association of appendiceal diverticulosis and neoplasia is known, it remains underemphasized in the teaching and practice of surgical pathology.
  • To investigate the frequency of appendiceal neoplasms with acquired diverticulosis, we conducted a retrospective analysis of all appendectomy specimens received in our institution for a 55-month period (January 2002-July 2006).
  • Eleven (48%) appendectomy specimens with diverticulosis also harbored an appendiceal neoplasm.
  • The association of appendiceal neoplasms with diverticulosis was statistically significant (P < .0001, 2-sided Fisher exact test).
  • Neoplastic processes included 5 well-differentiated neuroendocrine tumors (carcinoids), 3 mucinous adenomas, 1 tubular adenoma, and 2 adenocarcinomas.
  • We stress the need for meticulous gross assessment with histologic examination of the entire appendectomy specimen in cases of appendiceal diverticulosis.
  • Thorough examination is required to rule out an underlying neoplasm as a cause of diverticulosis.
  • As acquired diverticula represent a rare finding, examination of the entire appendix in this setting does not create a significant impact on the workload within the pathologic laboratory.
  • [MeSH-major] Adenocarcinoma / pathology. Appendiceal Neoplasms / pathology. Appendix / pathology. Carcinoid Tumor / pathology. Cystadenoma, Mucinous / pathology. Diverticulum / pathology

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  • (PMID = 18715614.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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6. Plöckinger U, Couvelard A, Falconi M, Sundin A, Salazar R, Christ E, de Herder WW, Gross D, Knapp WH, Knigge UP, Kulke MH, Pape UF, Frascati Consensus Conference participants: Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma. Neuroendocrinology; 2008;87(1):20-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma.
  • [MeSH-major] Appendiceal Neoplasms / therapy. Carcinoma / therapy. Consensus. Goblet Cells. Guidelines as Topic / standards

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  • [CommentIn] Neuroendocrinology. 2013;98(2):170 [24298561.001]
  • [CommentIn] Neuroendocrinology. 2013;98(2):170 [24135761.001]
  • (PMID = 17934252.001).
  • [ISSN] 1423-0194
  • [Journal-full-title] Neuroendocrinology
  • [ISO-abbreviation] Neuroendocrinology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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8. Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC, North American Neuroendocrine Tumor Society (NANETS): The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum. Pancreas; 2010 Aug;39(6):753-66
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  • [Title] The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum.
  • Well-differentiated neuroendocrine tumors (NETs) of the jejunum, ileum, and appendix are also collectively known as midgut carcinoids.
  • Classic carcinoid syndrome is more likely to appear in patients with advanced disease.
  • With the exception of small well-differentiated NETs of the appendix, NETs of the midgut have substantial risk of relapse after resection and need to be followed for at least 7 years.Metastatic/advanced NETs of the midgut are incurable.
  • Octreotide long-acting release has also recently been shown to delay disease progression.
  • [MeSH-major] Intestinal Neoplasms / diagnosis. Neuroendocrine Tumors / diagnosis
  • [MeSH-minor] Appendix / pathology. Cecum / pathology. Humans. Ileum / pathology. Jejunum / pathology

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  • (PMID = 20664473.001).
  • [ISSN] 1536-4828
  • [Journal-full-title] Pancreas
  • [ISO-abbreviation] Pancreas
  • [Language] eng
  • [Publication-type] Consensus Development Conference; Journal Article; Practice Guideline
  • [Publication-country] United States
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9. 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.
  • We therefore analyzed the expression of CDX-2, PDX-1, TTF-1, and neuroendocrine secretory protein-55 (NESP-55), a recently described member of the chromogranin family, in primary and metastatic WDNET.
  • 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.
  • Appendiceal carcinoids were uniformly positive for CDX-2 (11/11).
  • [MeSH-major] Biomarkers, Tumor / metabolism. Carcinoid Tumor / diagnosis. Gastrointestinal Neoplasms / diagnosis. Lung Neoplasms / diagnosis. Neoplasm Proteins / metabolism. Pancreatic Neoplasms / diagnosis
  • [MeSH-minor] DNA-Binding Proteins / metabolism. Diagnosis, Differential. Fluorescent Antibody Technique, Indirect. GTP-Binding Protein alpha Subunits, Gs / metabolism. Homeodomain Proteins / metabolism. Humans. Immunoenzyme Techniques. Trans-Activators / metabolism

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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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10. Rosato L, De Tomaa G, Mondini G, Bertone P, Fornari F, Orlassino R: [Neurendocrine tumours of the appendix, colon and rectum: current surgical aspects]. Chir Ital; 2007 Jul-Aug;59(4):445-52
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  • [Title] [Neurendocrine tumours of the appendix, colon and rectum: current surgical aspects].
  • [Transliterated title] Attualità nella chirurgia dei tumori neuroendocrini dell'appendice, del colon e del retto.
  • Neuroendocrine tumours (NET) are a heterogeneous group of neoplasms deriving from a system of diffuse neuroendocrine cells in organs and tissues, defined as the "diffuse neuroendocrine system".
  • Over the period from 1996 to 2005 42 patients with gastroenteropancreatic (GEP) NET were observed (M.F ratio: 1.5:1; mean age 58 years; > 60 years for all localisations except the appendix [< 39 years]).
  • Twenty-three were tumours of the appendix, colon and rectum, corresponding to 55% of all those affecting the digestive tract: 8 appendix (35%), 6 right colon (26%), 4 left colon (17%) and 5 rectum (22%).
  • The NET diagnosis was formulated in all cases on the basis of histological and immunohistochemical examinations.
  • In the RO-RI cases no relapses occurred and those who were not disease-free were treated with somatostatin analogues and/or chemotherapy.
  • NET of the appendix, colon and rectum are rare, despite being the most frequent among the GEP tumours, and are difficult to diagnose, and therefore sometimes pose problems of surgical therapy, which, when performed in time, may be curative.
  • NET of the appendix measuring <2 cm, localised in the distal part without local infiltration, can be treated by simple appendicectomy and removal of the mesenteriole; otherwise, right hemicolectomy is indicated.
  • The surgical treatment of tumours of the colon, except for well differentiated cases measuring <2 cm with a pedunculate structure such as to allow safe endoscopic removal, consists in radical hemicolectomy with lymphadenectomy.
  • Tumours measuring >2 cm or presenting muscular invasion and/or lymph-node metastases (malignant carcinoids), regardless of tumour diameter, are submitted to radical operations, as in the case of carcinoma of the rectum.
  • Extensive disease which is no longer curable with surgery alone is treated with chemotherapy and bio-chemotherapy, but it is above all treatment with somatostatin analogues that plays a major role in symptom control.
  • [MeSH-major] Appendectomy. Appendiceal Neoplasms / surgery. Colectomy. Colorectal Neoplasms / surgery. Neuroendocrine Tumors / surgery
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Follow-Up Studies. Hormones / therapeutic use. Humans. Lymph Node Excision. Lymphatic Metastasis. Male. Middle Aged. Minimally Invasive Surgical Procedures. Retrospective Studies. Somatostatin / analogs & derivatives. Somatostatin / therapeutic use. Treatment Outcome

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  • (PMID = 17966763.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Hormones; 51110-01-1 / Somatostatin
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11. 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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  • [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.
  • 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 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.
  • 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.
  • [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

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  • [CommentIn] Am J Surg Pathol. 2009 Aug;33(8):1259-60; author reply 1260-1 [19471156.001]
  • (PMID = 18685490.001).
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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12. Kinová S, Kekenák L, Kovácová E, Koren M: [Gastroenteropatic neuroendocrine tumors: multidisciplinary approach in therapy]. Vnitr Lek; 2010 Sep;56(9 Suppl):946-50

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Gastroenteropatic neuroendocrine tumors: multidisciplinary approach in therapy].
  • INTRODUCTION: Gastroenteropancreatic neuroendocrine tumors (GEP-NET) are classified on the basis of hormonal activity of tumor cells to functional and non-functional tumors.
  • Therapy of well differentiated NETs includes surgical procedures, debulking of tumor mass, biotherapy and peptid receptor radionuclid therapy.
  • AIM OF THE STUDY: Analysis of therapeutic modalities in group of patients with well differentiated GEP-NETs.
  • 1. 2010 we followed up 50 pts (19 men/31 women) with well differentiated GEP neuroendocrine tumors.
  • Primary localisation was: stomach--6 times, pancreas--9 times, duodenum--1 times, jejunum-- 4 times, appendix--3 times, ileum--23 times, rectum--4 times.
  • Metastatic disease was affirmed in time of diagnosis in 36 patients.
  • Carcinoid syndroma had 20 pts, 4 pts with pancreatic tumor had functional tumors (2 times overproduction of calcitonine, 1 times of gastrin, 1 times of insuline).
  • Surgical treatment was performed in 40 pts--resection of primary tumor and debulking of metastases, in 5 pts with pancreatic tumor resection was not possible due to invasion to sorrounding tissue and vessels.
  • Biological treatment with long acting somatostatin analogues was indicated in 20 pts with carcinoid syndroma and in 4 pts with functional pancreatic tumors.
  • In 5 pts with non resectable neuroendocrine carcinoma of pancreas peptid radionuclide receptor therapy (PRRT) was indicated: in 4 of them with 90Ytrium-DOTA-octreotid and in 1 patient with MIBG.
  • In all pts a reduction of tumor volume was noticed.
  • Biotherapy with somatostatin analogues reduced symptoms of hormonal activities and brought on stabilisation of disease in most of patients.
  • CONCLUSION: Complex therapy in patients with well differentiated neuroendocrine tumors markedly contributes to prolongation of survival of patients and also to enhancement quality of their life.
  • [MeSH-major] Gastrointestinal Neoplasms / therapy. Neuroendocrine Tumors / therapy

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  • (PMID = 21137165.001).
  • [ISSN] 0042-773X
  • [Journal-full-title] Vnitr̆ní lékar̆ství
  • [ISO-abbreviation] Vnitr Lek
  • [Language] slo
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Czech Republic
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13. Louthan O: [Neuroendocrine tumours of the appendix]. Vnitr Lek; 2009 Nov;55(11):1051-5

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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14. Kianmanesh R, O'toole D, Sauvanet A, Ruszniewski P, Belghiti J: [Surgical treatment of gastric, enteric, and pancreatic endocrine tumors Part 1. Treatment of primary endocrine tumors]. J Chir (Paris); 2005 May-Jun;142(3):132-49
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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 gastric, enteric, and pancreatic endocrine tumors Part 1. Treatment of primary endocrine tumors].
  • [Transliterated title] Traitement chirurgical des tumeurs endocrines gastro-entéro-pancréatiques.
  • Endocrine tumors (ET) of the digestive tract (formerly called neuroendocrine tumors) are rare.
  • They are classified into two principal types: gastrointestinal ET's (formerly called carcinoid tumors) which are the most common, and pancreaticoduodenal ET's.
  • Poorly-differentiated ET's have a poor prognosis and are treated by chemotherapy.
  • Surgical excision is the only curative treatment of well-differentiated ET's.
  • The surgical goals are to: 1. prolong survival by resecting the primary tumor and any nodal or hepatic metastases, 2. control the symptoms related to hormonal secretion, 3. prevent or treat local complications.
  • The most common sites of gastrointestinal ET's ( carcinoids) are the appendix and the rectum; these are often small (<1 cm), benign, and discovered fortuitously at the time of appendectomy or colonoscopic removal.
  • Ileal ET's, even if small, are malignant, frequently multiple, and complicated in 30-50% of cases by bowel obstruction, mesenteric invasion, or bleeding.
  • They are usually malignant and of advanced stage at diagnosis presenting as a palpable or obstructing mass or as liver metastases.
  • Insulinoma and gastrinoma (cause of the Zollinger-Ellison syndrome) are the most common functional ET's. 80% are sporadic; in these cases, tumor size, location, and malignant potential determine the type of resection which may vary from a simple enucleation to a formal pancreatectomy.
  • In 10-20% of cases, pancreaticoduodenal ET presents in the setting of multiple endocrine neoplasia (NEM type I), an autosomal-dominant genetic disease with multifocal endocrine involvement of the pituitary, parathyroid, pancreas, and adrenal glands.
  • For insulinoma with NEM-I, enucleation of lesions in the pancreatic head plus a caudal pancreatectomy is the most appropriate procedure.
  • For gastrinoma with NEM-I, the benefit of surgical resection for tumors less than 2-3 cm in size is not clear.
  • But the eventual development of liver metastases which are the most common cause of mortality still argues for an aggressive surgical approach in the early stages of the disease.
  • [MeSH-major] Carcinoid Tumor / surgery. Carcinoma, Islet Cell / surgery. Carcinoma, Neuroendocrine / surgery. Insulinoma / surgery. Intestinal Neoplasms / surgery. Multiple Endocrine Neoplasia Type 1 / surgery. Pancreatic Neoplasms / surgery. Stomach Neoplasms / surgery. Zollinger-Ellison Syndrome / surgery
  • [MeSH-minor] Adult. Gastrinoma / diagnosis. Gastrinoma / surgery. Glucagonoma / diagnosis. Glucagonoma / surgery. Humans. Liver Neoplasms / secondary. Lymphatic Metastasis. Malignant Carcinoid Syndrome / diagnosis. Malignant Carcinoid Syndrome / surgery. Multicenter Studies as Topic. Pancreatectomy. Postoperative Care. Postoperative Complications. Prognosis. Somatostatinoma / diagnosis. Somatostatinoma / surgery. Vipoma / diagnosis. Vipoma / surgery

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  • (PMID = 16142076.001).
  • [ISSN] 0021-7697
  • [Journal-full-title] Journal de chirurgie
  • [ISO-abbreviation] J Chir (Paris)
  • [Language] fre
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 236
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15. Niederhuber JE, Fojo T: Treatment of metastatic disease in patients with neuroendocrine tumors. Surg Oncol Clin N Am; 2006 Jul;15(3):511-33, viii
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of metastatic disease in patients with neuroendocrine tumors.
  • Gastroenteropancreatic neuroendocrine tumors (GENTs) compromise a heterogeneous group of relatively uncommon neoplasms with a yearly incidence rate of 1.2 to 3.0 per 100,000 population.
  • These tumors share numerous histologic and biologic features, allowing their consideration as a common entity.
  • They are postulated to arise from neuroendocrine cells, but most are not from neural crest origin.
  • Their predominant site of origin is the gastrointestinal tract, where most involve the small intestine and appendix, but are also found in the adrenal medulla, bronchopulmonary system, pancreas, thyroid, parathyroid, and paraganglia cells.
  • A common feature is their often indolent course, but some tumors are poorly differentiated and behave aggressively.
  • This article addresses the surgical management of endocrine malignancies and the treatment of metastatic disease in patients with neuroendocrine tumors.
  • [MeSH-major] Carcinoma, Neuroendocrine / secondary. Carcinoma, Neuroendocrine / therapy. Gastrointestinal Neoplasms / pathology. Gastrointestinal Neoplasms / therapy. Liver Neoplasms / surgery. Pancreatic Neoplasms / pathology. Pancreatic Neoplasms / therapy
  • [MeSH-minor] Algorithms. Antibiotics, Antineoplastic / administration & dosage. Antineoplastic Agents / administration & dosage. Chemoembolization, Therapeutic. Diagnostic Imaging. Hepatic Artery. Humans. Neoplasm Staging. Positron-Emission Tomography. Prognosis. Receptors, Somatostatin. Sensitivity and Specificity. Somatostatin / analogs & derivatives. Streptozocin / administration & dosage

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  • (PMID = 16882495.001).
  • [ISSN] 1055-3207
  • [Journal-full-title] Surgical oncology clinics of North America
  • [ISO-abbreviation] Surg. Oncol. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents; 0 / Receptors, Somatostatin; 51110-01-1 / Somatostatin; 5W494URQ81 / Streptozocin
  • [Number-of-references] 87
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16. Couvelard A: [Appendicular pathology. Mixed endocrine carcinoma with poorly differentiated large cell component]. Ann Pathol; 2010 Apr;30(2):124-9
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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. Mixed endocrine carcinoma with poorly differentiated large cell component].
  • [Transliterated title] Pathologie de l'appendice. Cas no 7. Carcinome endocrine mixte avec contingent peu différencié à grandes cellules.
  • [MeSH-major] Appendiceal Neoplasms / pathology. Appendicitis / etiology. Appendix / pathology. Carcinoma, Large Cell / pathology. Carcinoma, Neuroendocrine / pathology
  • [MeSH-minor] Adult. Antigens, CD56 / analysis. Appendectomy. Biomarkers, Tumor / analysis. Cell Differentiation. Female. Humans. Ki-67 Antigen / analysis. Lymphatic Metastasis. Neoplasm Proteins / analysis. Synaptophysin / analysis. Tumor Suppressor Protein p53 / analysis

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  • (PMID = 20451071.001).
  • [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 / Antigens, CD56; 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Neoplasm Proteins; 0 / Synaptophysin; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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