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3. Lambertini D, Bottini E, Talassi E, Tarchini R, Gaetti L, Bellomi A: [Acute renal failure caused by VIP-secreting tumor]. G Ital Nefrol; 2003 Jul-Aug;20(4):419-22
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  • [Transliterated title] Una causa rara di insufficienza renale acuta: il VIPoma.
  • Vipoma is a rare neuroendocrine tumor.
  • Metastases are present in 50% of patients at the time of diagnosis.
  • Treatment includes correction of volume, electrolyte, and metabolic abnormalities; CVVH during ARF; pharmacotherapy to decrease gastrointestinal secretion; and surgical resection of the vipoma.
  • [MeSH-major] Acute Kidney Injury / etiology. Pancreatic Neoplasms / complications. Vipoma / complications

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  • (PMID = 14523904.001).
  • [ISSN] 0393-5590
  • [Journal-full-title] Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
  • [ISO-abbreviation] G Ital Nefrol
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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4. Cellier C, Yaghi C, Cuillerier E, Siauve N, Berger A, Carnot F, Haddad C, Barbier JP, Landi B: Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil. Am J Gastroenterol; 2000 Jan;95(1):289-93
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  • [Title] Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil.
  • The VIPoma syndrome is rare.
  • Somatostatin analogs and interferon-a can be helpful in the symptomatic control of the disease, but the efficacy of chemotherapy in metastatic disease is limited.
  • We report the case of a 32-yr-old patient who had a primary intestinal VIPoma with peritoneal carcinomatosis and hepatic metastases.
  • Somatostatin analogs and conventional chemotherapy regimens were not effective on VIPoma syndrome and tumor progression.
  • The combination of 5- fluorouracil and interferon-alpha was associated with a major clinical improvement and tumor regression.
  • Further investigations should evaluate the place of such a combination as a first line treatment for patients with metastatic neuroendocrine tumors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Jejunal Neoplasms / pathology. Vipoma / drug therapy. Vipoma / secondary
  • [MeSH-minor] Adult. Fluorouracil / administration & dosage. Humans. Interferon-alpha / administration & dosage. Liver Neoplasms / drug therapy. Liver Neoplasms / radiography. Liver Neoplasms / secondary. Male. Peritoneal Neoplasms / drug therapy. Peritoneal Neoplasms / radiography. Peritoneal Neoplasms / secondary. Tomography, X-Ray Computed

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  • (PMID = 10638600.001).
  • [ISSN] 0002-9270
  • [Journal-full-title] The American journal of gastroenterology
  • [ISO-abbreviation] Am. J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Interferon-alpha; U3P01618RT / Fluorouracil
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5. Rammer M, Kirchgatterer A, Höbling W, Stockhammer M, Knoflach P: [W.D.H.A. Syndrome due to occult neuroendocrine malignancy with concomitant liver metastases]. Z Gastroenterol; 2003 Feb;41(2):185-9
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  • [Transliterated title] Okkulte, maligne neuroendokrine Neoplasie mit Lebermetastasen als Ursache eines WDHA-Syndroms.
  • The biopsy of the liver shows a malignant neuroendocrine tumour.
  • In the absence of clinical symptoms a wait and see procedure with clinical and imaging controls at regular intervals is arranged.
  • The existence of secretory diarrhoea, hypokalaemia and hypercalcaemia arouses suspicion of vipoma.
  • A vipoma is a rare differential diagnosis of secretory diarrhoea.
  • This case report describes the remarkable constellation of liver metastases of a malignant neuroendocrine neoplasm without a primary tumour and the clinical presentation of a W.D.H.A. syndrome (watery diarrhoea, hypokalaemia and hypo- or achlorhydria).
  • Despite extensive disease, therapy with octreotide and prednisolone provides a good clinical response.
  • [MeSH-major] Liver Neoplasms / secondary. Neoplasms, Unknown Primary / diagnosis. Vipoma / secondary
  • [MeSH-minor] Biopsy, Needle. Diagnosis, Differential. Diagnostic Imaging. Humans. Liver / pathology. Male. Middle Aged. Octreotide / administration & dosage. Prednisolone / administration & dosage. Radioligand Assay. Receptors, Somatostatin / analysis. Vasoactive Intestinal Peptide / blood. Water-Electrolyte Balance / drug effects

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  • (PMID = 12592602.001).
  • [ISSN] 0044-2771
  • [Journal-full-title] Zeitschrift für Gastroenterologie
  • [ISO-abbreviation] Z Gastroenterol
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Receptors, Somatostatin; 37221-79-7 / Vasoactive Intestinal Peptide; 9PHQ9Y1OLM / Prednisolone; RWM8CCW8GP / Octreotide
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6. Remme CA, de Groot GH, Schrijver G: Diagnosis and treatment of VIPoma in a female patient. Eur J Gastroenterol Hepatol; 2006 Jan;18(1):93-9
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  • [Title] Diagnosis and treatment of VIPoma in a female patient.
  • We report a case of VIPoma in an 83-year-old female patient, who presented with frequent and excessive diarrhoea, muscle weakness, and severe hypokalaemia.
  • Abdominal computed tomography (CT) revealed a 4x6 cm mass in the body of the pancreas.
  • The patient was treated with a long-acting octreotide, after which her symptoms diminished.
  • The VIPoma syndrome is caused by a neuroendocrine tumour, usually located in the pancreas, which secretes VIP, causing severe diarrhoea, dehydration and hypokalaemia.
  • Treatment options include resection of the tumour, chemotherapy or the reduction of symptoms with somatostatin analogues.
  • We provide an overview of the incidence, pathophysiology, diagnosis, treatment strategies, and prognosis of this rare syndrome.
  • [MeSH-major] Pancreatic Neoplasms / radiography. Vipoma / radiography
  • [MeSH-minor] Aged, 80 and over. Antineoplastic Agents, Hormonal / therapeutic use. Female. Follow-Up Studies. Humans. Octreotide / therapeutic use. Tomography, X-Ray Computed

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  • (PMID = 16357627.001).
  • [ISSN] 0954-691X
  • [Journal-full-title] European journal of gastroenterology & hepatology
  • [ISO-abbreviation] Eur J Gastroenterol Hepatol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; RWM8CCW8GP / Octreotide
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7. Rigabert J, De Clermont H: [Diagnostic procedures and more particularly, place of scintigraphy in neuroendocrine tumors, example of vipoma in MEN 1]. Ann Endocrinol (Paris); 2007 Jun;68(2-3):199-203
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  • [Title] [Diagnostic procedures and more particularly, place of scintigraphy in neuroendocrine tumors, example of vipoma in MEN 1].
  • [Transliterated title] Outils diagnostiques et plus particulièrement, place de la scintigraphie dans les tumeurs neuroendocrines: l'exemple d'un vipome dans une NEM de type 1.
  • Functioning endocrine pancreatic tumors in multiple endocrine neoplasia type 1 (MEN1) are rare.
  • This case is interesting in many ways: this is an exceptional illustration of MEN 1 with vipoma associated with calcitonin secretion and it is also a good example of the benefits and limitations of each diagnostic procedure in the heterogeneous group of neuroendocrine tumors.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / diagnosis. Neuroendocrine Tumors / diagnosis. Vipoma / diagnosis
  • [MeSH-minor] Adult. Biomarkers. Calcitonin / metabolism. Female. Humans. Hypercalcemia / drug therapy. Hyperparathyroidism / etiology. Magnetic Resonance Imaging. Technetium Tc 99m Sestamibi. Tomography, X-Ray Computed

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  • (PMID = 17292846.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Biomarkers; 9007-12-9 / Calcitonin; 971Z4W1S09 / Technetium Tc 99m Sestamibi
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8. Shorter NA, Glick RD, Klimstra DS, Brennan MF, Laquaglia MP: Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present. J Pediatr Surg; 2002 Jun;37(6):887-92
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  • [Title] Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present.
  • BACKGROUND: Malignant tumors of the pancreas are uncommon in children and adolescents and only recently have the most common tumor types been well characterized.
  • As a result, the treatment approach to these patients has yet to be standardized, and much of the information available in the literature, particularly with regard to the role of chemotherapy and radiation, is anecdotal.
  • METHODS: A retrospective review was undertaken of all patients less than 21 years of age with malignant pancreatic tumors who were cared for at Memorial Sloan-Kettering since 1967.
  • The pathologic types were pancreatoblastoma, 5; solid pseudopapillary tumor, 7; acinar cell carcinoma, 1; nonfunctioning pancreatic endocrine neoplasm, 1; malignant VIPoma, 1; and PNET, 2.
  • Chemotherapy or radiation were used in selected cases.
  • CONCLUSIONS: Unlike malignant pancreatic tumors in adults, tumors in children and adolescents usually are resectable, and long-term survival is likely.
  • The roles of chemotherapy and radiation remain undefined.
  • [MeSH-major] Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Carcinoma, Acinar Cell / pathology. Carcinoma, Acinar Cell / therapy. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Liver Neoplasms / secondary. Lung Neoplasms / secondary. Male. Neoplasm Recurrence, Local. Pancreatectomy. Radiotherapy, Adjuvant. Retrospective Studies. Risk Assessment. Treatment Outcome. Vipoma / pathology. Vipoma / therapy

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  • [Copyright] Copyright 2002, Elsevier Science (USA). All rights reserved.
  • (PMID = 12037756.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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9. Halászlaki C, Horváth H, Kiss L, Takács I, Speer G, Nagy Z, Winternitz T, Dabasi G, Zalatnai A, Patócs A, Lakatos P: [Verner-Morrison syndrome: a case study]. Orv Hetil; 2010 Jul 4;151(27):1111-4
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  • Typical symptoms play a momentous role in the diagnosis of VIPoma.
  • Imaging modalities include endoscopic ultrasonography, computed tomography and magnetic resonance imaging, and particularly, scintigraphy with somatostatin analogues.
  • Treatment options include resection of the tumor, chemotherapy or the reduction of symptoms with somatostatin analogues.
  • VIPoma cases may be associated with multiple endocrine neoplasia type 1.
  • [MeSH-major] Pancreatic Neoplasms. Vipoma
  • [MeSH-minor] Achlorhydria / etiology. Aged. Biomarkers, Tumor / metabolism. Diarrhea / etiology. Endosonography. Female. Humans. Hypokalemia / etiology. Immunohistochemistry. Magnetic Resonance Imaging. Multiple Endocrine Neoplasia Type 1 / complications. Tomography, X-Ray Computed. Vasoactive Intestinal Peptide / metabolism

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  • (PMID = 20558361.001).
  • [ISSN] 0030-6002
  • [Journal-full-title] Orvosi hetilap
  • [ISO-abbreviation] Orv Hetil
  • [Language] hun
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Hungary
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 37221-79-7 / Vasoactive Intestinal Peptide
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10. Berković MC, Altabas V, Herman D, Hrabar D, Goldoni V, Vizner B, Zjacić-Rotkvić V: A single-centre experience with octreotide in the treatment of different hypersecretory syndromes in patients with functional gastroenteropancreatic neuroendocrine tumors. Coll Antropol; 2007 Jun;31(2):531-4
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  • [Title] A single-centre experience with octreotide in the treatment of different hypersecretory syndromes in patients with functional gastroenteropancreatic neuroendocrine tumors.
  • The aim of this research was to assess the clinical and biochemical efficacy of the octreotide in the treatment of patients with various functional gastroenteropancreatic neuroendocrine tumors (GEP-NETs).
  • They were diagnosed with VIPoma, glucagonoma, gastrinoma, medullary thyroid carcinoma (solitary and as a part of MEN-II syndrome), pancreatic carcinoids (solitary and as a part of multiple endocrine neoplasia type-1 syndrome-MEN-1 syndrome) and midgut carcinoids.
  • All had a metastatic disease at the time of diagnosis and a positive octreoscan finding.
  • Symptomatic efficacy assessments were made by diarrhea reductions during treatment course, and laboratory efficacy was assessed through changes in 5-HIAA and CgA levels.
  • Assessments were made initially and following 6 months of therapy.
  • There was a positive correlation between the 5-HIAA reduction and the decrease in stool number at baseline and during treatment course (p < 0.05).
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Neuroendocrine Tumors / drug therapy. Octreotide / therapeutic use. Pancreatic Neoplasms / drug therapy. Stomach Neoplasms / drug therapy. Thyroid Neoplasms / drug therapy
  • [MeSH-minor] Biomarkers, Tumor. Female. Humans. Male. Malignant Carcinoid Syndrome / drug therapy. Middle Aged. Treatment Outcome

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  • (PMID = 17847934.001).
  • [ISSN] 0350-6134
  • [Journal-full-title] Collegium antropologicum
  • [ISO-abbreviation] Coll Antropol
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Croatia
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Biomarkers, Tumor; RWM8CCW8GP / Octreotide
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11. Song S, Shi R, Li B, Liu Y: Diagnosis and treatment of pancreatic vasoactive intestinal peptide endocrine tumors. Pancreas; 2009 Oct;38(7):811-4
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  • [Title] Diagnosis and treatment of pancreatic vasoactive intestinal peptide endocrine tumors.
  • METHOD: Clinical manifestations, laboratory examination, imaging features, surgical findings, and pathological findings of 4 patients with VIPoma admitted in our hospital from 1991 to the present are discussed.
  • Two patients with hepatic metastases received a combination therapy of octreotide, surgery, and chemotherapy, which resulted in symptom improvement and normalization of the serum potassium values.
  • Distal pancreatic resection and second resection of hepatic metastatic lesions were performed in 1 patient.
  • CONCLUSIONS: Typical symptoms play an important role in the diagnosis of VIPoma.
  • Octreotide therapy has advanced the preoperative electrolyte management, and the combination of octreotide, chemotherapy, and surgery may be helpful in metastatic disease.
  • [MeSH-major] Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / therapy. Vipoma / diagnosis. Vipoma / therapy

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  • (PMID = 19657309.001).
  • [ISSN] 1536-4828
  • [Journal-full-title] Pancreas
  • [ISO-abbreviation] Pancreas
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromogranin A; 37221-79-7 / Vasoactive Intestinal Peptide
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12. Corbetta S, Peracchi M, Cappiello V, Lania A, Lauri E, Vago L, Beck-Peccoz P, Spada A: Circulating ghrelin levels in patients with pancreatic and gastrointestinal neuroendocrine tumors: identification of one pancreatic ghrelinoma. J Clin Endocrinol Metab; 2003 Jul;88(7):3117-20
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  • We measured plasma ghrelin levels in 16 patients with gastrointestinal carcinoid (10 with midgut and 6 with gastric carcinoid), 24 patients with pancreatic tumor (8 with gastrinoma, 2 with insulinoma, 2 with vipoma, 1 with glucagonoma, and 11 with nonfunctioning tumor), and 35 healthy controls.
  • Plasma ghrelin levels recorded in patients with gastroenteropancreatic tumors were similar to controls (mean +/- SE, 182.7 +/- 66.5 pM in patients vs. 329 +/- 32 pM in controls, P = not significant), and no significant difference between gastrointestinal and pancreatic, functioning and nonfunctioning, and metastatic and nonmetastatic tumors was observed.
  • One patient with metastatic nonfunctioning pancreatic tumor had circulating ghrelin levels of 12,000 pM that were slightly reduced during chemotherapy and interferon therapy.
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / blood. Female. Gastrinoma / blood. Ghrelin. Glucagonoma / blood. Humans. Insulinoma / blood. Male. Middle Aged. Retrospective Studies. Vipoma / blood

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  • (PMID = 12843152.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ghrelin; 0 / Peptide Hormones
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13. Oda Y, Tanaka Y, Naruse T, Sasanabe R, Tsubamoto M, Funahashi H: Expression of somatostatin receptor and effects of somatostatin analog on pancreatic endocrine tumors. Surg Today; 2002;32(8):690-4
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  • METHODS: Somatostatin receptor subtypes 1, 2, and 3 (sst 1, 2, and 3) were immunohistochemically investigated in seven pancreatic endocrine tumors: four insulinomas, one VIPoma, and two nonfunctioning tumors associated with multiple endocrine neoplasia type I, using paraffin sections.
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Octreotide / therapeutic use. Pancreatic Neoplasms / drug therapy. Pancreatic Neoplasms / metabolism. Receptors, Somatostatin / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Humans. Immunohistochemistry. Insulinoma / drug therapy. Insulinoma / metabolism. Male. Middle Aged. Multiple Endocrine Neoplasia Type 1 / drug therapy. Multiple Endocrine Neoplasia Type 1 / metabolism. Vipoma / drug therapy. Vipoma / metabolism

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  • (PMID = 12181718.001).
  • [ISSN] 0941-1291
  • [Journal-full-title] Surgery today
  • [ISO-abbreviation] Surg. Today
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Receptors, Somatostatin; 0 / somatostatin receptor 2; 0 / somatostatin receptor 3; RWM8CCW8GP / Octreotide
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14. Nikou GC, Toubanakis C, Nikolaou P, Giannatou E, Safioleas M, Mallas E, Polyzos A: VIPomas: an update in diagnosis and management in a series of 11 patients. Hepatogastroenterology; 2005 Jul-Aug;52(64):1259-65
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  • BACKGROUND/AIMS: VIPoma is a rare pancreatic endocrine tumor (PET) which secretes excessive amounts of VIP (Vasoactive Intestinal Peptide) that causes a special clinical syndrome characterized by secretory diarrhea, hypokalemia and achlorhydria.
  • Among a total number of 76 patients (pts) with PETs, we present in this study 11 pts with VIPoma syndrome focusing on our diagnostic and therapeutic approach, in parallel with a brief review of the literature.
  • VIP levels at the time of diagnosis were more than 3 or 10 times the upper normal limit in 7/11 (63.6%) or 4/11 (36.4%) pts, respectively.
  • A surgical resection was possible in 7/11 (63.6%) pts, while pts with metastatic disease already or poorly differentiated tumors also received additional treatment with somatostatin analogues and chemotherapy.
  • Also, surgical treatment, as extensive as possible, in combination with somatostatin analogues or chemotherapy when necessary, may also result in prolonged survival, also in patients with advanced disease.
  • [MeSH-major] Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / therapy. Vipoma / diagnosis. Vipoma / therapy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents, Hormonal / therapeutic use. Child, Preschool. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Octreotide / therapeutic use. Pancreatectomy. Survival Rate

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  • (PMID = 16001675.001).
  • [ISSN] 0172-6390
  • [Journal-full-title] Hepato-gastroenterology
  • [ISO-abbreviation] Hepatogastroenterology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; RWM8CCW8GP / Octreotide
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15. Dadan J, Wojskowicz P, Wojskowicz A: Neuroendocrine tumors of the pancreas. Wiad Lek; 2008;61(1-3):43-7
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  • The most often NET of the pancreas are insulinoma, gastrinoma, glucagonoma, somatostatinoma, VIPoma.
  • Those tests are useful in monitoring treatment and in prognostication course of the disease.
  • Imaging methods especially useful in localization GEP-NET are: ultrasound (US), endoscopic ultrasound (EUS), somatostatin receptor scintigraphy (SRS), computer tomography (CT), magnetic resonance (MR) and angiography.
  • Surgical treatment depends on progression of disease as well as on localization of tumor and consists in both radical methods and palliative operations.
  • The gold standard in pharmacological treatment are somatostatin analogs which can induce long-term remission even in inoperable lesions.
  • Although NET of pancreas are very rare. they are still important diagnostic and therapeutic problem and requires interdisciplinary co-operation.
  • [MeSH-major] Carcinoma, Islet Cell / diagnosis. Carcinoma, Islet Cell / therapy. Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / therapy
  • [MeSH-minor] Gastrinoma / diagnosis. Gastrinoma / metabolism. Gastrinoma / therapy. Glucagonoma / diagnosis. Glucagonoma / metabolism. Glucagonoma / therapy. Humans. Insulinoma / diagnosis. Insulinoma / metabolism. Insulinoma / therapy. Somatostatinoma / diagnosis. Somatostatinoma / metabolism. Somatostatinoma / therapy. Vipoma / diagnosis. Vipoma / metabolism. Vipoma / therapy

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  • (PMID = 18717042.001).
  • [ISSN] 0043-5147
  • [Journal-full-title] Wiadomości lekarskie (Warsaw, Poland : 1960)
  • [ISO-abbreviation] Wiad. Lek.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Poland
  • [Number-of-references] 30
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17. 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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  • [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.
  • 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 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.
  • [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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18. Desai KK, Khan MS, Toumpanakis C, Caplin ME: Management of gastroentero-pancreatic neuroendocrine tumors (GEP-NETs). Minerva Gastroenterol Dietol; 2009 Dec;55(4):425-43
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  • The therapeutic options which are reviewed, including the use of somatostatin analogues, the role of surgery, the use of chemotherapy, biotherapy using interferon, peptide receptor targeted therapy.
  • Authors have focused on the newest therapeutic modalities, e.g., radionuclide peptide receptor targeted therapy with Yttrium-90 and Lutetium-177, the newest somatostatin analogues such as pasireotide and angiogenic inhibitors.
  • In conclusion, with the increasing number of investigative procedures and therapeutic options available to diagnose and treat carcinoid tumors, it is vital to have a multidisciplinary approach.
  • Furthermore, additional scientific research and controlled clinical trials are needed to determine the efficacy of the many treatment options, which for these rare tumors can only be achieved by collaboration.
  • [MeSH-major] Carcinoid Tumor / therapy. Neuroendocrine Tumors / therapy. Pancreatic Neoplasms / therapy
  • [MeSH-minor] Angiogenesis Inhibitors / therapeutic use. Biochemistry. Embolization, Therapeutic. Gastrinoma / therapy. Hepatic Artery. Humans. Insulinoma / therapy. Liver Transplantation. Malignant Carcinoid Syndrome / therapy. Multiple Endocrine Neoplasia Type 1 / complications. Patient Selection. Receptors, Peptide / physiology. Somatostatin / analogs & derivatives. Somatostatin / therapeutic use. Vipoma / therapy. von Hippel-Lindau Disease / complications

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  • (PMID = 19942827.001).
  • [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
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Receptors, Peptide; 51110-01-1 / Somatostatin; 98H1T17066 / pasireotide
  • [Number-of-references] 160
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19. Arnold C: [Neuroendocrine tumors of the gastrointestinal tract]. Praxis (Bern 1994); 2007 Jan 10;96(1-2):19-28
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  • [Transliterated title] Neuroendokrine Tumoren des Gastrointestinaltraktes.
  • Diazoxid can further inhibit insulin secretion, proton pump inhibitors are the therapy of choice for acid hypersecretion in Zollinger-Ellison syndrome.
  • Advanced neuroendocrine cancers can be treated with chemotherapy.
  • Recently, radio receptor therapy with 90Y-DOTA Octreotid and 177Lu-DOTA Octreotate was established in advanced neuroendocrine cancers and is further evaluated in studies.
  • An overview about epidemiology, clinical features, diagnostic methods and therapy of NET of the gastrointestinal tract will is provided in this article.
  • [MeSH-minor] Antineoplastic Agents, Hormonal / therapeutic use. Biological Therapy. Chromogranin A / analysis. Clinical Trials as Topic. Clinical Trials, Phase II as Topic. Clinical Trials, Phase III as Topic. Diagnosis, Differential. Gastrinoma / diagnosis. Gastrinoma / therapy. Gastrointestinal Agents / therapeutic use. Glucagonoma / diagnosis. Glucagonoma / therapy. Humans. Incidence. Insulinoma / diagnosis. Insulinoma / therapy. Malignant Carcinoid Syndrome / diagnosis. Malignant Carcinoid Syndrome / therapy. Multiple Endocrine Neoplasia Type 1 / diagnosis. Octreotide / therapeutic use. Prevalence. Proton Pump Inhibitors. Receptors, Somatostatin / analysis. Vipoma / diagnosis. Vipoma / therapy. World Health Organization. Zollinger-Ellison Syndrome / diagnosis. Zollinger-Ellison Syndrome / therapy. von Hippel-Lindau Disease / diagnosis

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  • (PMID = 17256557.001).
  • [ISSN] 1661-8157
  • [Journal-full-title] Praxis
  • [ISO-abbreviation] Praxis (Bern 1994)
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Chromogranin A; 0 / Gastrointestinal Agents; 0 / Proton Pump Inhibitors; 0 / Receptors, Somatostatin; RWM8CCW8GP / Octreotide
  • [Number-of-references] 26
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20. Ruszniewski P, Ish-Shalom S, Wymenga M, O'Toole D, Arnold R, Tomassetti P, Bax N, Caplin M, Eriksson B, Glaser B, Ducreux M, Lombard-Bohas C, de Herder WW, Delle Fave G, Reed N, Seitz JF, Van Cutsem E, Grossman A, Rougier P, Schmidt W, Wiedenmann B: Rapid and sustained relief from the symptoms of carcinoid syndrome: results from an open 6-month study of the 28-day prolonged-release formulation of lanreotide. Neuroendocrinology; 2004;80(4):244-51
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  • This 6-month, open, non-controlled, multicenter, dose-titration study evaluated the efficacy and safety of 28-day prolonged-release (PR) lanreotide in the treatment of carcinoid syndrome.
  • Six treatments of 28-day PR lanreotide were administered by deep subcutaneous injection.
  • Flushing decreased from a mean of 3.0 at baseline to 2.3 on day 1, and 2.0 on day 2, with a daily mean of 2.1 for the first week post-treatment (p < 0.05).
  • Diarrhea decreased from a mean of 5.0 at baseline to 4.3 on day 1 (p < 0.05), and 4.5 on day 2, with a daily mean of 4.4 for the first week post-treatment (p < 0.001).
  • Treatment was well tolerated.
  • [MeSH-major] Delayed-Action Preparations / therapeutic use. Malignant Carcinoid Syndrome / drug therapy. Peptides, Cyclic / therapeutic use. Somatostatin / analogs & derivatives. Somatostatin / therapeutic use
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Chromogranins / blood. Dose-Response Relationship, Drug. Drug Administration Schedule. Drug Evaluation / methods. Female. Flushing / drug therapy. Flushing / physiopathology. Humans. Hydroxyindoleacetic Acid / urine. Male. Middle Aged. Quality of Life. Time Factors. Treatment Outcome. Vipoma / drug therapy. Vipoma / physiopathology

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  • [CommentIn] Neuroendocrinology. 2005;81(3):137-8 [15976511.001]
  • (PMID = 15627802.001).
  • [ISSN] 0028-3835
  • [Journal-full-title] Neuroendocrinology
  • [ISO-abbreviation] Neuroendocrinology
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Clinical Trial, Phase III; Comparative Study; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Chromogranins; 0 / Delayed-Action Preparations; 0 / Peptides, Cyclic; 118992-92-0 / lanreotide; 51110-01-1 / Somatostatin; 54-16-0 / Hydroxyindoleacetic Acid
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21. Schoevaerdts D, Favet L, Zekry D, Sieber CC, Michel JP: Vipoma: effective treatment with octreotide in the oldest old. J Am Geriatr Soc; 2001 Apr;49(4):496-7
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  • [Title] Vipoma: effective treatment with octreotide in the oldest old.
  • [MeSH-major] Antidiarrheals / therapeutic use. Antineoplastic Agents, Hormonal / therapeutic use. Octreotide / therapeutic use. Pancreatic Neoplasms / drug therapy. Vipoma / drug therapy

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  • (PMID = 11347804.001).
  • [ISSN] 0002-8614
  • [Journal-full-title] Journal of the American Geriatrics Society
  • [ISO-abbreviation] J Am Geriatr Soc
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antidiarrheals; 0 / Antineoplastic Agents, Hormonal; RWM8CCW8GP / Octreotide
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