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1. Shojamanesh H, Gibril F, Louie A, Ojeaburu JV, Bashir S, Abou-Saif A, Jensen RT: Prospective study of the antitumor efficacy of long-term octreotide treatment in patients with progressive metastatic gastrinoma. Cancer; 2002 Jan 15;94(2):331-43
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  • [Title] Prospective study of the antitumor efficacy of long-term octreotide treatment in patients with progressive metastatic gastrinoma.
  • BACKGROUND: Malignant pancreatic endocrine tumors (PETs) have a poor prognosis and existing antitumor treatments are unsatisfactory.
  • Recent studies have shown somatostatin analogues to have antitumor growth effects in patients with malignant PETs; however, to the authors' knowledge, little information exists regarding their efficacy or effect on survival in patients with progressive malignant gastrinoma, the most common symptomatic malignant PET.
  • The purpose of the current study was to study prospectively the efficacy, safety, and effect on survival of long-term treatment with octreotide in consecutive patients with progressive malignant gastrinoma.
  • METHODS: Fifteen consecutive patients with malignant gastrinoma with progressive hepatic metastases were studied.
  • All patients underwent conventional imaging studies (computed tomography scan, magnetic resonance imaging, ultrasound, and, if needed, selective angiography) and somatostatin receptor scintigraphy prior to treatment and at 3-6-month intervals while receiving treatment.
  • Tumor size and/or number were used to classify patient responses as either no tumor response or tumor response (stabilization or decrease in size).
  • Treatment response was correlated with tumor and clinical characteristics.
  • RESULTS: Tumors in 8 of the 15 patients studied (53%) responded at 3 months, with 47% (7 of 15 patients) demonstrating tumor stabilization and 6% (1 of 15 patients) demonstrating a decrease in tumor size.
  • Six of the eight responders were continuing to respond at the time of last follow-up.
  • Tumor response did not correlate with any clinical parameter (e.g., tumor extent, fasting gastrin, or acid secretory rates).
  • However, slow-growing tumors were more likely to respond prior to treatment (86% vs. 0%) (P < 0.0014).
  • Two patients (13%) developed serious side effects that required the withdrawal of octreotide.
  • CONCLUSIONS: Octreotide is an effective antitumor treatment in patients with progressive malignant gastrinoma.
  • In approximately 50% of these patients octreotide has an antigrowth effect; treatment is associated with a low incidence of serious side effects compared with other antitumor treatments commonly used and, in contrast to many studies, the growth response is long-lasting.
  • The results of the current study suggest that octreotide treatment should replace chemotherapy as the standard treatment for these patients, especially those patients with slow-growing tumors.
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Gastrinoma / drug therapy. Liver Neoplasms / drug therapy. Octreotide / therapeutic use. Pancreatic Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Disease Progression. Female. Follow-Up Studies. Gastrins / metabolism. Humans. Lymphatic Metastasis. Male. Middle Aged. Neoplasm Recurrence, Local. Predictive Value of Tests. Prospective Studies. Receptors, Somatostatin / metabolism. Time Factors. Tomography, Emission-Computed, Single-Photon. Treatment Outcome

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  • (PMID = 11900219.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Gastrins; 0 / Receptors, Somatostatin; RWM8CCW8GP / Octreotide
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2. Wong H, Yau T, Chan P, Ng IO, Chan G, Hui P, Law WL, Lo CM, Hedley AJ, Epstein RJ: PPI-delayed diagnosis of gastrinoma: oncologic victim of pharmacologic success. Pathol Oncol Res; 2010 Mar;16(1):87-91
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  • [Title] PPI-delayed diagnosis of gastrinoma: oncologic victim of pharmacologic success.
  • Functional neuroendocrine tumors are often low-grade malignant neoplasms that can be cured by surgery if detected early, and such detection may in turn be accelerated by the recognition of neuropeptide hypersecretion syndromes.
  • Uniquely, however, relief of peptic symptoms induced by hypergastrinemia is now available from acid-suppressive drugs such as proton-pump inhibitors (PPIs).
  • Here we describe a clinical case in which time to diagnosis from the onset of peptic symptoms was delayed more than 10 years, in part reflecting symptom masking by continuous prescription of the PPI omeprazole.
  • We propose diagnostic criteria for this under-recognized new clinical syndrome, and recommend that physicians routinely measure serum gastrin levels in persistent cases of PPI-dependent dyspepsia unassociated with H. pylori.
  • [MeSH-major] Anti-Ulcer Agents / therapeutic use. Delayed Diagnosis / adverse effects. Gastrinoma / pathology. Gastroesophageal Reflux / drug therapy. Omeprazole / therapeutic use. Pancreatic Neoplasms / secondary
  • [MeSH-minor] Appendectomy. Barrett Esophagus / complications. Barrett Esophagus / drug therapy. Humans. Hypertension / complications. Immunohistochemistry. Liver Neoplasms / secondary. Male. Middle Aged

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  • (PMID = 19693706.001).
  • [ISSN] 1532-2807
  • [Journal-full-title] Pathology oncology research : POR
  • [ISO-abbreviation] Pathol. Oncol. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Anti-Ulcer Agents; KG60484QX9 / Omeprazole
  • [Other-IDs] NLM/ PMC2953631
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3. Ehehalt F, Saeger HD, Schmidt CM, Grützmann R: Neuroendocrine tumors of the pancreas. Oncologist; 2009 May;14(5):456-67
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  • PNETs are rare neoplasms, compared with carcinomas arising from pancreatic exocrine tissue.
  • They, like other neuroendocrine tumor types, display variable malignant potential, hormone-related syndromes (functionality), localization, and genetic background.
  • Although tumor origin and molecular pathogenesis remain poorly understood, recently established grading and staging systems facilitate patient risk stratification, and thereby directly impact clinical decision making.
  • Although the optimal clinical management of PNETs involves a multidisciplinary approach, surgery remains the only curative treatment for early-stage disease.
  • Alternative therapeutic approaches applied to PNETs, including chemotherapy, radiofrequency ablation, transarterial chemoembolization, biotherapy, polypeptide radionuclide receptor therapy, antiangiogenic therapy, and selective internal radiotherapy, have failed to demonstrate a long-term survival benefit.
  • Surgery remains the primary therapeutic option for patients with PNETs.
  • Research on PNETs is desperately needed to improve the therapeutic options for patients with this disease.
  • [MeSH-minor] Gastrinoma / diagnosis. Gastrinoma / therapy. Humans. Incidence. Insulinoma / diagnosis. Insulinoma / therapy. Neoplasm Staging. Prognosis

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  • (PMID = 19411317.001).
  • [ISSN] 1549-490X
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 130
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4. Caplin ME, Mielcarek W, Buscombe JR, Jones AL, Croasdale PL, Cooper MS, Burroughs AK, Hilson AW: Toxicity of high-activity 111In-Octreotide therapy in patients with disseminated neuroendocrine tumours. Nucl Med Commun; 2000 Jan;21(1):97-102
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  • [Title] Toxicity of high-activity 111In-Octreotide therapy in patients with disseminated neuroendocrine tumours.
  • Disseminated neuroendocrine tumours are difficult to treat and are generally not responsive to radiotherapy or chemotherapy.
  • Nuclear medicine techniques using a radiolabelled somatostatin analogue, 111In-Octreotide, have been used for the diagnosis of neuroendocrine tumours.
  • It has been suggested that high activities of such an agent may have a therapeutic effect.
  • Eight patients with known disseminated neuroendocrine tumours were enrolled in the study; six had carcinoid tumours, one had a medullary cell carcinoma of the thyroid and one patient had a malignant gastrinoma.
  • Tests of vital signs, renal, liver and endocrine function as well as haematological markers were taken before and after treatment.
  • The treatment was well tolerated with only one patient suffering from a sensation of flushing during the infusion but no changes in vital sings.
  • There was a transient (up to 48 h) drop in circulating lymphocytes in four patients and platelets in two patients; no supportive therapy was needed.
  • [MeSH-major] Carcinoid Tumor / radiotherapy. Gastrinoma / radiotherapy. Multiple Endocrine Neoplasia / radiotherapy. Neuroendocrine Tumors / radiotherapy. Octreotide / analogs & derivatives. Pentetic Acid / analogs & derivatives. Radiopharmaceuticals / adverse effects

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  • (PMID = 10717909.001).
  • [ISSN] 0143-3636
  • [Journal-full-title] Nuclear medicine communications
  • [ISO-abbreviation] Nucl Med Commun
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 142694-57-3 / SDZ 215-811; 7A314HQM0I / Pentetic Acid; RWM8CCW8GP / Octreotide
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5. Yang J, Zhou GW, Chen X, Wei Y, Peng CH, Ning G, Li HW: [Diagnosis and treatment of multiple endocrine neoplasia type 1 related pancreatic endocrine tumors]. Zhonghua Wai Ke Za Zhi; 2009 Mar 1;47(5):329-32
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  • [Title] [Diagnosis and treatment of multiple endocrine neoplasia type 1 related pancreatic endocrine tumors].
  • OBJECTIVE: To summarize the experience on diagnosis and treatment of multiple endocrine neoplasia type 1 (MEN-1) related pancreatic endocrine tumors (PET).
  • Drug therapy, surgery and follow-up were applied on the patients.
  • RESULTS: There were 9 patients having insulinomas including 2 cases of multiple insulinomas and 1 case presenting an insulinoma, multiple nonfunctional PET and malignant duodenum gastrinoma with liver metastasis.
  • Twelve insulinomas, four nonfunctional PET and one duodenum gastrinoma were found in the operations.
  • CONCLUSIONS: Well recognizing PET and MEN-1, early diagnosing MEN-1 related PET, appropriately surgical intervention will prove patients' life quality and will help for prolonging patients' survival time.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / diagnosis. Multiple Endocrine Neoplasia Type 1 / surgery. Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / surgery


6. Schott M, Scherbaum WA, Feldkamp J: [Drug therapy of endocrine neoplasms. Part II: Malignant gastrinomas, insulinomas, glucagonomas, carcinoids and other tumors]. Med Klin (Munich); 2000 Feb 15;95(2):81-4
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  • [Title] [Drug therapy of endocrine neoplasms. Part II: Malignant gastrinomas, insulinomas, glucagonomas, carcinoids and other tumors].
  • [Transliterated title] Medikamentöse Therapie endokriner Karzinome. Teil II: Maligne Gastrinome, Insulinome, Glukagonome, Karzinoide und andere Tumoren.
  • TREATMENT: Because of the rarity and missing prospective studies as well as radiotherapy and chemotherapy resistance of these tumors, generally accepted conventional therapy guidelines for these endocrine carcinomas do not exist.
  • Surgery and radionucleotide treatment should be considered as first line therapy.
  • Chemotherapy is usually not effective.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Gastrointestinal Neoplasms / drug therapy. Neoplasms, Hormone-Dependent / drug therapy
  • [MeSH-minor] Antineoplastic Agents, Hormonal / therapeutic use. Carcinoid Tumor / drug therapy. Dose-Response Relationship, Drug. Gastrinoma / drug therapy. Glucagonoma / drug therapy. Humans. Insulinoma / drug therapy. Octreotide / therapeutic use. Somatostatin / analogs & derivatives

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  • (PMID = 10714123.001).
  • [ISSN] 0723-5003
  • [Journal-full-title] Medizinische Klinik (Munich, Germany : 1983)
  • [ISO-abbreviation] Med. Klin. (Munich)
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] GERMANY
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Hormonal; 51110-01-1 / Somatostatin; RWM8CCW8GP / Octreotide
  • [Number-of-references] 49
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7. Park SY, Rhee Y, Youn JC, Park YN, Lee S, Kim DM, Song SY, Lim SK: Ectopic Cushing's syndrome due to concurrent corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) secreted by malignant gastrinoma. Exp Clin Endocrinol Diabetes; 2007 Jan;115(1):13-6
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  • [Title] Ectopic Cushing's syndrome due to concurrent corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) secreted by malignant gastrinoma.
  • Further work-up revealed high gastrin levels and abdominal computed tomography (CT) scans showed a large pancreatic head mass with multiple liver metastases.
  • Since an operation was not feasible, chemotherapy was conducted using paclitaxel and etoposide.
  • These two drugs were chosen according to the IN VITRO chemotherapy response assay to maximize the treatment.
  • This report demonstrates concurrent ACTH- and CRH-related ectopic Cushing's syndrome caused by malignant gastrinoma with multiple liver metastases that was treated with marginal success using a multidisciplinary medical approach.
  • [MeSH-major] Adrenocorticotropic Hormone / blood. Corticotropin-Releasing Hormone / blood. Cushing Syndrome / blood. Duodenal Neoplasms / secretion. Gastrinoma / secretion. Liver Neoplasms / secretion. Pancreatic Neoplasms / secretion
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Etoposide / administration & dosage. Female. Humans. Hydrocortisone / blood. Paclitaxel / administration & dosage

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  • (PMID = 17286228.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 9002-60-2 / Adrenocorticotropic Hormone; 9015-71-8 / Corticotropin-Releasing Hormone; P88XT4IS4D / Paclitaxel; WI4X0X7BPJ / Hydrocortisone
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8. Granberg D, Jacobsson H, Oberg K, Gustavsson J, Lehtihet M: Regression of a large malignant gastrinoma on treatment with Sandostatin LAR: a case report. Digestion; 2008;77(2):92-5
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  • [Title] Regression of a large malignant gastrinoma on treatment with Sandostatin LAR: a case report.
  • The gastrin overproduction leads to the Zollinger-Ellison syndrome with multiple gastric and duodenal ulcers and diarrhea.
  • About two thirds of gastrinomas are malignant.
  • Diagnosis is made by clinical history, gastroscopy, and measurement of serum gastrin, gastric juice pH, CT scan, endoscopic ultrasonography and somatostatin receptor scintigraphy.
  • Medical therapy for tumor control includes biotherapy with alpha-interferon and somatostatin analogs yielding a response rate of about 10-15%, chemotherapy or targeted radiotherapy.
  • We describe a patient with almost complete response on treatment with Sandostatin LAR, a long-acting somatostatin analog.
  • In patients with metastatic gastrinomas not suitable for chemotherapy, interferon or targeted radiotherapy, single therapy with somatostatin analogs may be an alternative.
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Gastrinoma / drug therapy. Octreotide / therapeutic use. Pancreatic Neoplasms / drug therapy
  • [MeSH-minor] Female. Humans. Middle Aged. Treatment Outcome

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  • [Copyright] Copyright 2008 S. Karger AG, Basel.
  • (PMID = 18376130.001).
  • [ISSN] 1421-9867
  • [Journal-full-title] Digestion
  • [ISO-abbreviation] Digestion
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; RWM8CCW8GP / Octreotide
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9. Wang L, Zhao YP, Lee CI, Liao Q: Diagnosis and treatment of malignant pancreatic endocrine tumour. Chin Med Sci J; 2004 Jun;19(2):130-3
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  • [Title] Diagnosis and treatment of malignant pancreatic endocrine tumour.
  • OBJECTIVE: To summarize our experience in the diagnosis and treatment of malignant pancreatic endocrine tumour.
  • METHODS: We retrospectively reviewed 36 cases of malignant pancreatic endocrine tumours in our hospital from July 1987 to April 2002, and summarized its clinical features.
  • RESULTS: Liver metastasis was the main malignant manifestation of malignant pancreatic endocrine tumours (incidence rate 72.2%).
  • Removals of primary lesion and isolated hepatic metastatic lesion were means of curative therapy.
  • Interventional chemotherapy was an important adjuvant treatment.
  • CONCLUSION: Comprehensive therapy plays an important role in improving the prognosis of malignant pancreatic endocrine tumour.

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  • (PMID = 15250251.001).
  • [ISSN] 1001-9294
  • [Journal-full-title] Chinese medical sciences journal = Chung-kuo i hsueh k'o hsueh tsa chih
  • [ISO-abbreviation] Chin. Med. Sci. J.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] China
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10. Ducreux M, Baudin E, Schlumberger M: [Treatment strategy of neuroendocrine tumors]. Rev Prat; 2002 Feb 1;52(3):290-6
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  • [Title] [Treatment strategy of neuroendocrine tumors].
  • [Transliterated title] Stratégie de traitement des tumeurs neuro-endocrines.
  • Therapeutic strategy of neuroendocrine tumours is complex, due to their heterogeneity and to the fact that although generally slow growing, a significant proportion demonstrates aggressive tumour growth.
  • Surgery remains the mainstay of treatment if the tumour can be resected.
  • Metastatic pancreatic neuroendocrine tumour are treated when resection is not feasible with combination chemotherapy using adriamycin and streptozotocin, which remains a standard of care.
  • In well differentiated tumour of the gut or the lung there is no clear standard of chemotherapy and treatment vary according to the tumour course.
  • In indolent cases, somatostatin analogues are the best treatment, in case of aggressive tumours chemoembolisation should be preferred when the disease is located or predominant in the liver.
  • Poorly differentiated tumours are treated by combination chemotherapy with etoposide and cisplatin, and surgery has no indication.
  • Gastrinoma and other pancreatic tumours arising in the context of multiple endocrine neoplasia type I disease need a specific therapeutic strategy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Growth Hormone / therapeutic use. Hormones / therapeutic use. Neuroendocrine Tumors / drug therapy. Pancreatic Neoplasms / drug therapy
  • [MeSH-minor] Antibiotics, Antineoplastic / therapeutic use. Chemoembolization, Therapeutic. Doxorubicin / therapeutic use. Gastrinoma / drug therapy. Humans. Malignant Carcinoid Syndrome / drug therapy. Multiple Endocrine Neoplasia. Neoplasm Metastasis. Streptozocin / therapeutic use

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  • (PMID = 11925720.001).
  • [ISSN] 0035-2640
  • [Journal-full-title] La Revue du praticien
  • [ISO-abbreviation] Rev Prat
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Hormones; 5W494URQ81 / Streptozocin; 80168379AG / Doxorubicin; 9002-72-6 / Growth Hormone
  • [Number-of-references] 23
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11. Oberg K, Eriksson B: Endocrine tumours of the pancreas. Best Pract Res Clin Gastroenterol; 2005 Oct;19(5):753-81
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  • Some of the tumours may be part of multiple endocrine neoplasia type one (MEN-1) syndrome or von Hippel-Lindau (vHL) disease.
  • The biochemical diagnosis of EPT is based on hormones and amines released.
  • Besides specific markers such as insulin, there are also general tumour markers such as chromogranin A, which is the most valuable marker and has been reported to be increased in plasma in 50-80% of patients with EPTs and correlates with tumour burden.
  • The location of endocrine tumours of the pancreas includes different techniques, from endoscopic investigations to scintigraphy (e.g. somatostatin receptor scintigraphy) and positron emission tomography.
  • The medical treatment of endocrine pancreatic tumours consists of chemotherapy, somatostatin analogues and alpha-interferon.
  • None of these can cure a patient with malignant disease.
  • In future, therapy will be custom-made and based on current knowledge of tumour biology and molecular genetics.
  • [MeSH-major] Carcinoma, Neuroendocrine / drug therapy. Carcinoma, Neuroendocrine / pathology. Pancreatic Neoplasms / drug therapy. Pancreatic Neoplasms / pathology
  • [MeSH-minor] Biopsy, Needle. Female. Gastrinoma / drug therapy. Gastrinoma / epidemiology. Gastrinoma / pathology. Glucagonoma / drug therapy. Glucagonoma / epidemiology. Glucagonoma / pathology. Humans. Immunohistochemistry. Incidence. Insulinoma / drug therapy. Insulinoma / epidemiology. Insulinoma / pathology. Male. Molecular Biology. Neoplasm Staging. Prognosis. Risk Assessment. Somatostatinoma / drug therapy. Somatostatinoma / epidemiology. Somatostatinoma / pathology. Survival Rate. Treatment Outcome. Zollinger-Ellison Syndrome / drug therapy. Zollinger-Ellison Syndrome / epidemiology. Zollinger-Ellison Syndrome / pathology

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  • (PMID = 16253899.001).
  • [ISSN] 1521-6918
  • [Journal-full-title] Best practice & research. Clinical gastroenterology
  • [ISO-abbreviation] Best Pract Res Clin Gastroenterol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 141
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12. Campana D, Piscitelli L, Mazzotta E, Bonora M, Serra C, Salomone L, Corinaldesi R, Tomassetti P: Zollinger-Ellison syndrome. Diagnosis and therapy. Minerva Med; 2005 Jun;96(3):187-206
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  • [Title] Zollinger-Ellison syndrome. Diagnosis and therapy.
  • Zollinger-Ellison syndrome (ZES) is characterised by refractory peptic ulcer disease, severe diarrhoea and gastric acid hypersecretion associated with an islet-cell tumor of the pancreas (gastrinoma).
  • ZES is sporadic in 62-80% of cases and in 20-38% of cases is associated with multiple endocrine neoplasia type 1 (MEN 1).
  • The diagnosis of ZES is certain when the plasma gastrin is >1000 pg/mL and the basal acid output is >15 mEq/h in patients with an intact stomach, >5 mEq/h in gastrectomised patients, or when the hypergastrinemia is associated with a pH <2.
  • Treatment is based on the control of gastric acid hypersecretion and of the malignant tumor and its possible metastases.
  • Proton pump inhibitors are the most effective antisecretory drugs and can be administered at high dosages without drug-related adverse effects.
  • When liver metastases are also present, their debulking may improve symptoms and survival, and facilitate medical treatment.
  • Somatostatin analogues can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric enterochromaffin-like cells, and can thus contribute to treating the disease more effectively.
  • Chemotherapy and/or interferon are indicated only in patients with malignant progressive disease.
  • [MeSH-major] Zollinger-Ellison Syndrome / diagnosis. Zollinger-Ellison Syndrome / therapy
  • [MeSH-minor] Biomarkers / blood. Gastrinoma / pathology. Gastrins / blood. Humans

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  • (PMID = 16175161.001).
  • [ISSN] 0026-4806
  • [Journal-full-title] Minerva medica
  • [ISO-abbreviation] Minerva Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Gastrins
  • [Number-of-references] 0
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13. Tomassetti P, Salomone T, Migliori M, Campana D, Corinaldesi R: Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients. Drugs Aging; 2003;20(14):1019-34
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  • [Title] Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients.
  • Zollinger-Ellison syndrome is characterised by refractory peptic ulcer disease, severe diarrhoea and gastric acid hypersecretion associated with an islet-cell tumour of the pancreas (gastrinoma).
  • Zollinger-Ellison syndrome is sporadic in 62-80% of cases and in 20-38% of cases is associated with multiple endocrine neoplasia type 1 (MEN 1).
  • The diagnosis of Zollinger-Ellison syndrome is certain when the plasma gastrin is >1000 pg/mL and the basal acid output is >15 mEq/h in patients with an intact stomach, >5 mEq/h in gastrectomised patients, or when this hypergastrinemia is associated with a pH <2.
  • The treatment is based on control of gastric acid hypersecretion and of the malignant tumour and its possible metastases.
  • Proton pump inhibitors are the most effective antisecretory drugs and can be administered in the elderly at high dosages without drug-related adverse effects.
  • As an initial therapy, daily dosages of omeprazole 80-100 mg or pantoprazole 40-160 mg are employed.
  • In long-term treatment the doses can be greatly reduced once effective control of the gastric output has been established.
  • Intravenous proton pump inhibitors may be administered when patients cannot take oral therapy, particularly in acute conditions.
  • When liver metastases are also present, their debulking may improve symptoms and survival, and facilitate medical treatment.
  • Somatostatin analogues can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric enterochromaffin-like (ECL) cells and can thus contribute to treating the disease more effectively.
  • Chemotherapy is not the therapy of choice in patients with gastrinomas and is indicated only in those with malignant progressive disease; interferon alpha, embolisation and chemoembolisation are not advisable for the elderly.
  • The treatment of elderly Zollinger-Ellison syndrome patients, similarly to all elderly oncological patients, should be based on the use of comprehensive geriatric assessment.
  • This will enable the clinician to define the functional status of the elderly person, to decide whether the patient can tolerate surgery and/or the stress of antineoplastic therapy, and finally, to determine whether this patient can tolerate an aggressive treatment for Zollinger-Ellison syndrome or whether the only possible choice is palliative relief of symptoms.
  • [MeSH-major] Geriatrics. Helicobacter pylori. Histamine H2 Antagonists / therapeutic use. Proton Pump Inhibitors. Zollinger-Ellison Syndrome
  • [MeSH-minor] Aged. Carcinoid Tumor / complications. Helicobacter Infections / complications. Humans. Multiple Endocrine Neoplasia Type 1 / complications

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  • (PMID = 14651442.001).
  • [ISSN] 1170-229X
  • [Journal-full-title] Drugs & aging
  • [ISO-abbreviation] Drugs Aging
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Histamine H2 Antagonists; 0 / Proton Pump Inhibitors
  • [Number-of-references] 143
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14. Norton JA: Endocrine tumours of the gastrointestinal tract. Surgical treatment of neuroendocrine metastases. Best Pract Res Clin Gastroenterol; 2005 Aug;19(4):577-83
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  • [Title] Endocrine tumours of the gastrointestinal tract. Surgical treatment of neuroendocrine metastases.
  • Neuroendocrine tumors of the intestinal tract have low malignant potential but can result in decreased survival if they spread to the liver.
  • The estimated 5-year survival of patients with liver metastases from neuroendocrine tumor is only 20%.
  • Treatment options for liver neuroendocrine tumor include long-acting somatostatin receptor antagonists (LAR), inteferon-alpha, chemotherapy and hepatic artery embolisation with and without chemotherapy.
  • Aggressive major surgery for liver neuroendocrine tumor metastases can be performed safely with acceptable mortality by experienced surgeons.
  • Results have been similar for patients with gastrinoma and pancreatic neuroendocrine tumors.
  • It should be the first-line therapy for patients with liver neuroendocrine tumors in whom the tumor can be completely removed.
  • [MeSH-minor] Gastrinoma / diagnosis. Gastrinoma / secondary. Gastrinoma / surgery. Hepatectomy. Humans

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  • (PMID = 16183528.001).
  • [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] 24
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15. 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 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.
  • [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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16. Fjällskog ML, Sundin A, Westlin JE, Oberg K, Janson ET, Eriksson B: Treatment of malignant endocrine pancreatic tumors with a combination of alpha-interferon and somatostatin analogs. Med Oncol; 2002;19(1):35-42
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  • [Title] Treatment of malignant endocrine pancreatic tumors with a combination of alpha-interferon and somatostatin analogs.
  • Somatostatin analogs and alpha-interferon induce good responses as single drugs in the treatment of endocrine pancreatic tumors.
  • All patients except one had received prior treatment and were in a progressive state.
  • All three patients previously progressing on both alpha-interferon and somatostatin analog as single drugs achieved a stabilization of the disease when treated with the combination (median duration 10 mo).
  • Seven of eight (88%) patients previously progressing on alpha-interferon treatment benefited from the combination with biochemical partial response or stabilization.
  • All six patients previously progressing during somatostatin analog treatment achieved biochemical partial response or stabilization.
  • More than 80% of patients who progressed during previous treatment with either drug benefited from the combined treatment, which also was well tolerated.
  • Thus, a combination of alpha-interferon and somatostatin analogs may be considered for patients previously progressing on treatment with alpha-interferon or somatostatin analogs.
  • However, in this study, the value of sequential treatment has not been evaluated.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Gastrinoma / drug therapy. Glucagonoma / drug therapy. Pancreatic Neoplasms / drug therapy. Somatostatin / analogs & derivatives
  • [MeSH-minor] Adult. Aged. Female. Humans. Interferon-alpha / administration & dosage. Male. Middle Aged. Octreotide / administration & dosage. Peptides, Cyclic / administration & dosage. Tomography, Emission-Computed

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  • (PMID = 12025889.001).
  • [ISSN] 1357-0560
  • [Journal-full-title] Medical oncology (Northwood, London, England)
  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Interferon-alpha; 0 / Peptides, Cyclic; 0G3DE8943Y / lanreotide; 51110-01-1 / Somatostatin; RWM8CCW8GP / Octreotide
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17. Viola KV, Sosa JA: Current advances in the diagnosis and treatment of pancreatic endocrine tumors. Curr Opin Oncol; 2005 Jan;17(1):24-7
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  • [Title] Current advances in the diagnosis and treatment of pancreatic endocrine tumors.
  • PURPOSE OF REVIEW: A comprehensive literature review of more than 200 original manuscripts published in the last 18 months was conducted to summarize landmark studies performed on the molecular biology, diagnosis, imaging, and treatment of endocrine tumors of the pancreas.
  • New imaging, including F-dopa positron emission tomography and laparoscopic ultrasound, and the effective combination of existing modalities localized and staged tumors with greater accuracy.
  • Nonoperative treatment strategies show promise; discovery of the antiangiogenic properties of octreotide and the overexpression of tyrosine kinase receptors such as c-kit, epidermal growth factor receptor, and platelet-derived growth factor receptor on malignant endocrine pancreatic tumors may lead to promising pharmacologic treatment.
  • [MeSH-major] Gastrinoma / diagnosis. Gastrinoma / drug therapy. Insulinoma / diagnosis. Insulinoma / drug therapy. Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / drug therapy
  • [MeSH-minor] Biomarkers, Tumor / blood. Chromogranin A. Chromogranins / blood. Humans. Incidence. Multiple Endocrine Neoplasia Type 1 / epidemiology. Multiple Endocrine Neoplasia Type 1 / pathology

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  • (PMID = 15608508.001).
  • [ISSN] 1040-8746
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromogranin A; 0 / Chromogranins
  • [Number-of-references] 14
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18. Dall'igna P, Cecchetto G, Bisogno G, Conte M, Chiesa PL, D'Angelo P, De Leonardis F, De Salvo G, Favini F, Ferrari A, TREP Group: Pancreatic tumors in children and adolescents: the Italian TREP project experience. Pediatr Blood Cancer; 2010 May;54(5):675-80
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  • INTRODUCTION: Malignant pancreatic tumors are exceedingly rare in pediatric age and their clinical features and treatment usually go unappreciated by most pediatric oncologists and surgeons.
  • RESULTS: Tumor types were 4 pancreatoblastomas, 2 pancreatic carcinomas, 3 neoplasms of the endocrine pancreas, and 12 solid pseudopapillary tumors.
  • Three of the four patients with pancreatoblastoma had advanced disease at diagnosis and were given chemotherapy; at the time of this report, three patients were alive in first remission, while one died due to treatment toxicity.
  • Both the cases of pancreatic carcinoma had the acinar cell subtype and successfully underwent pancreaticoduodenectomy with complete tumor resection, remaining without evidence of disease at the time of this analysis.
  • The histological diagnoses of the three endocrine tumors were a malignant islet cell tumor, a gastrinoma, and a well-differentiated tumor.
  • All 12 patients with solid pseudopapillary tumors underwent complete tumor resection and were given no adjuvant treatment; 11 were alive in first remission, while one experienced a local and distant relapse 5 years after diagnosis.
  • CONCLUSIONS: Surgery remains the keystone of treatment for pancreatic tumors in pediatric age as in adults.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Italy / epidemiology. Male. Prospective Studies. Treatment Outcome

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  • [CommentIn] Pediatr Blood Cancer. 2010 May;54(5):659-60 [20063425.001]
  • (PMID = 19998473.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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19. 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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  • Although generally slow growing, a significant minority demonstrate aggressive tumor growth.
  • 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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20. Alexakis N, Neoptolemos JP: Pancreatic neuroendocrine tumours. Best Pract Res Clin Gastroenterol; 2008;22(1):183-205
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  • Except for insulinoma all types are malignant in >50% of cases.
  • In contrast approximately 50% gastrinomas and the majority of non-functioning pancreatic neuroendocrine tumours are malignant.
  • Surgical excision is a key aspect of treatment for all cases of sporadic gastrinoma and if >2.5 cm in MEN1.
  • The local treatment for liver metastases is now well established and options include liver resection, chemoembolisation and radiofrequency ablation.
  • Systemic therapies have also been better defined and include radionuclide therapy against somatostatin receptors or MIBG and chemotherapy especially for poorly differentiated tumours.
  • [MeSH-minor] 3-Iodobenzylguanidine / therapeutic use. Antineoplastic Agents / therapeutic use. Hormones / therapeutic use. Humans. Liver Neoplasms / secondary. Neoplasm Staging. Positron-Emission Tomography / methods. Receptors, Somatostatin / therapeutic use. Somatostatin / analogs & derivatives. Somatostatin / therapeutic use. Tomography, X-Ray Computed / methods. von Hippel-Lindau Disease / complications

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  • (PMID = 18206821.001).
  • [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
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Hormones; 0 / Receptors, Somatostatin; 35MRW7B4AD / 3-Iodobenzylguanidine; 51110-01-1 / Somatostatin
  • [Number-of-references] 125
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21. 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.
  • The recently published WHO-classification grouped the tumors according to their tumor size, angioinvasion and Ki-67 index.
  • NET are mainly diagnosed in an advanced tumor stadium because of the paucity of symptoms or when symptoms occur due to endocrine hypersecretion.
  • 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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22. 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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