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1. Shaib W, Mitchell K, Saif MW: Amelioration of symptoms and reduction of VIP levels after hepatic artery chemoembolization in a patient with sandostatin resistant VIPoma. Yale J Biol Med; 2010 Mar;83(1):27-33
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  • [Title] Amelioration of symptoms and reduction of VIP levels after hepatic artery chemoembolization in a patient with sandostatin resistant VIPoma.
  • Vasoactive intestinal polypeptide secreting islet cell tumors (VIPomas) are neuroendocrine tumors that secrete excessive amounts of vasoactive intestinal polypeptide (VIP) that cause distinct syndromes characterized by large-volume diarrhea, hypokalemia, and dehydration.
  • The annual incidence of these tumors is estimated to be about one per 10,000,000 individuals in the general population.
  • We report a successful treatment of VIPoma with hepatic chemoembolization of a metastatic hepatic lesion evidenced by a reduction of VIP levels and resolutions of symptoms in a patient with pancreatic VIPoma unresponsive to increased doses of an octreotide analog.
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Embolization, Therapeutic. Hepatic Artery / surgery. Octreotide / therapeutic use. Vasoactive Intestinal Peptide / metabolism. Vipoma
  • [MeSH-minor] Aged, 80 and over. Female. Humans. Pancreatic Neoplasms / drug therapy. Pancreatic Neoplasms / pathology. Pancreatic Neoplasms / surgery. Treatment Outcome

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  • (PMID = 20351979.001).
  • [ISSN] 1551-4056
  • [Journal-full-title] The Yale journal of biology and medicine
  • [ISO-abbreviation] Yale J Biol Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 37221-79-7 / Vasoactive Intestinal Peptide; RWM8CCW8GP / Octreotide
  • [Other-IDs] NLM/ PMC2844690
  • [Keywords] NOTNLM ; VIPoma / hepatic artery chemoembolization
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2. 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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  • [Title] VIPomas: an update in diagnosis and management in a series of 11 patients.
  • 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.
  • The diagnosis was based upon compatible clinical features and serum VIP values and was supported by the estimation of other peptides and neuroendocrine markers such as gastrin, pancreatic polypeptide and chromogranin-A (CgA).
  • 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.
  • Liver metastases and poor differentiation of tumors seemed to be negative prognostic factors.
  • 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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3. 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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  • [Title] [Verner-Morrison syndrome: a case study].
  • [Transliterated title] Verner-Morrison-szindróma egy esete.
  • Verner and Morrison described a syndrome of watery diarrhea, hypokalemia, and achlorhydria (WDHA) in 1958.
  • VIPomas producing high amounts of vasoactive intestinal peptide (VIP) commonly originate from the pancreas.
  • Typical symptoms play a momentous role in the diagnosis of VIPoma.
  • Morbidity from untreated WDHA syndrome is associated with long-standing dehydration and with electrolyte and acid-base metabolism disorders, which may cause chronic renal failure.
  • Assessment of specific marker (VIP) offers high sensitivity in establishing the diagnosis.
  • 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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4. Lecorguillé M, Hammel P, Couvelard A, O'Toole D, Ratouis A, Belghiti J, Lévy P, Ruszniewski P: [Jejunal vipoma]. Gastroenterol Clin Biol; 2004 Aug-Sep;28(8-9):797-800
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  • [Title] [Jejunal vipoma].
  • [Transliterated title] Localisation jéjunale d'un vipome.
  • We report here case of a 57-year-old woman presenting with a metastatic vipoma revealed by secretory diarrhea and severe ionic disorders successfully treated by somatostatin administration.
  • The primitive tumour, located in the jejunum, was identified peroperatively.
  • Both the primitive lesion and the liver metastases were resected at the same time.
  • Early tumour relapse occurred and was unsuccessfully treated by systemic chemotherapy (5-fluorouracil, streptozotocin and doxorubin) and chemoembolization.
  • [MeSH-major] Jejunal Neoplasms / diagnosis. Vipoma / diagnosis

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  • (PMID = 15646540.001).
  • [ISSN] 0399-8320
  • [Journal-full-title] Gastroentérologie clinique et biologique
  • [ISO-abbreviation] Gastroenterol. Clin. Biol.
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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5. 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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  • [Title] [Acute renal failure caused by VIP-secreting tumor].
  • [Transliterated title] Una causa rara di insufficienza renale acuta: il VIPoma.
  • A 74-year-old woman had secretory diarrhea, severe metabolic acidosis, hypokalemia, hypovolemia, and acute renal failure caused by a pancreatic vasoactive intestinal polypeptide (VIP)-secreting tumor.
  • Vipoma is a rare neuroendocrine tumor.
  • Diagnosis requires the documentation of large volumes of secretory diarrhea, elevated VIP plasma levels, and the localization of the VIP-secreting 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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6. Simonenko VB, Dulin PA, Makanin MA: [Somatostatin analogues in treatment of gastrointestinal and pancreatic neuroendocrine tumors]. Klin Med (Mosk); 2006;84(4):4-8
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  • [Title] [Somatostatin analogues in treatment of gastrointestinal and pancreatic neuroendocrine tumors].
  • For these reasons, synthetic analogues of somatostatin, among which sandostatin (octreotide acetate) was the first one, were developed.
  • Other cyclic analogues with similar sensitivity and activity profile, such as lanreotide (somatulin), somatostatin-14, and SOM 230, have been developed as well.
  • Sandostatin therapy is indicated to patients with functionally active neuroendocrine tumors of the stomach, duodenum, small bowel, or appendix.
  • Glucagonomas, vipomas, and, to a lesser degree, gastrinomas and metastatic insulinomas are examples of functionally active endocrine pancreatic tumors that should be treated with sandostatin.
  • Other syndromes, which should be treated with octreotide, include ectopic secretion of adrenocorticotropic hormone in Cushing syndrome, oncogenic osteomalacia, and hypercalciemia resulting from ectopic secretion of parathyroid-like peptide.
  • In patients with an advanced carcinoid syndrome, the starting dose of sandostatin (ocreotide) is 150 mcgr administered three times a day in hypordermic injections during 10 to 14 days, after which sandostatin LAR is administered in a dose of 20 mg once a month.
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Gastrointestinal Neoplasms / drug therapy. Neuroendocrine Tumors / drug therapy. Pancreatic Neoplasms / drug therapy. Somatostatin / analogs & derivatives

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  • (PMID = 16755846.001).
  • [ISSN] 0023-2149
  • [Journal-full-title] Klinicheskaia meditsina
  • [ISO-abbreviation] Klin Med (Mosk)
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Russia (Federation)
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 51110-01-1 / Somatostatin
  • [Number-of-references] 25
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7. Libé R, Chanson P: [Endocrine tumors of the pancreas (EPTs) in multiple endocrine neoplasia (MEN1): up-date on prognostic factors, diagnostic procedures and treatment]. Ann Endocrinol (Paris); 2007 Jun;68 Suppl 1:1-8
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  • [Title] [Endocrine tumors of the pancreas (EPTs) in multiple endocrine neoplasia (MEN1): up-date on prognostic factors, diagnostic procedures and treatment].
  • [Transliterated title] Les tumeurs endocrines du pancréas lors de la néoplasie endocrinienne multiple type 1 (NEM1): actualités sur les facteurs pronostiques, l'imagerie et le traitement.
  • Endocrine pancreatic tumors (EPTs) are uncommon tumors, representing 1-2% of all pancreatic neoplasms.
  • They are categorized on the basis of their clinical features into functioning and non-functioning tumors.
  • EPTs may be part of the multiple endocrine neoplasia type 1 (MEN 1), an autosomal dominant syndrome due to inactivating germline mutation of the menin gene.
  • The most prevalent are the gastrinomas (20-60%), then the insulinomas (5-10%), the glucagonamas and VIPomas (6-10%), whereas the nonfunctioning EPTs are present in 20-40% of patients.
  • Among the different imaging techniques, echoendoscopy, computed tomography (CT) and magnetic resonance imaging (MRI) are indicated for the detection of the primary tumor, but (III)In-octreotide scintigraphy has the highest sensitivity for detecting metastases.
  • The choice of treatment is still debated and is different when the tumor occurs as a part of the MEN syndrome.
  • The surgical treatment is the first choice for insulinomas and is more controversial for gastrinomas.
  • The medical treatment includes somatostatin analogues (SA), chemotherapy and interferon-alpha (IFN-alpha).
  • SA seem to improve the symptoms and have an antiproliferative effect, the most striking effect being seen in patients with VIPomas.
  • Chemotherapy, which is generally proposed as a combination of streptozotocin (STZ) and 5-fluorouracil (5-FU) or doxorubicin, is indicated when the tumors tend to grow.
  • Interferon-alpha (IFN-alpha) stimulates the immune system, blocks the tumor cells in the G1/S-phase of the cell cycle, inhibits protein and hormone synthesis and inhibits angionenesis.
  • Treatment with IFN has been shown to produce symptomatic response in 40-60% of patients, a biochemical response in 30-60% and tumor shrinkage in 10-15%.
  • [MeSH-major] Carcinoma, Islet Cell. Multiple Endocrine Neoplasia Type 1
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Prognosis

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  • [ErratumIn] Ann Endocrinol (Paris). 2008 Nov;69(5):459-60
  • (PMID = 17961653.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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8. 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.
  • OBJECTIVE: To discuss our experience in diagnosing and treating pancreatic vasoactive intestinal peptide-secreting tumors (VIPomas) by summarizing clinical information of 4 patients.
  • 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.
  • The pancreatic body and tail were the main locations of lesions.
  • Two tumors were shown in the pancreatic body and tail in 1 patient.
  • 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.
  • Resection of the pancreatic body and tail was done in 1 patient, and pancreatoduodenectomy was performed in another 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
  • [MeSH-minor] Adult. Chromogranin A / metabolism. Diagnosis, Differential. Fatal Outcome. Female. Humans. Immunohistochemistry. Male. Middle Aged. Vasoactive Intestinal Peptide / metabolism

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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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9. 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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  • [Title] [W.D.H.A. Syndrome due to occult neuroendocrine malignancy with concomitant liver metastases].
  • [Transliterated title] Okkulte, maligne neuroendokrine Neoplasie mit Lebermetastasen als Ursache eines WDHA-Syndroms.
  • The biopsy of the liver shows a malignant neuroendocrine tumour.
  • Further diagnostic investigation including gastroscopy, colonoscopy, enteroclysis, thoracal and abdominal CT and somatostatin-receptor-scintigraphy does not localise the primary 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.
  • This is proven by a remarkably elevated plasma concentration of vasoactive intestinal peptide (VIP).
  • Once more, an accurate investigation is started but no primary tumour can be discovered despite extensive liver metastases.
  • 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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10. 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.
  • We present a case of a symptomatic neuroendocrine tumor in a 27-year old woman.
  • The identification of the nature of the neuroendocrine tumors was difficult despite the use of a wide range of diagnostic procedures.
  • 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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11. 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.
  • It is usually caused by a neuroendocrine tumor located in the pancreas.
  • 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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12. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • Laboratory analysis showed elevated levels of both vasoactive intestinal polypeptide (VIP; 153 pmol/l) and pancreatic polypeptide (161 pmol/l).
  • In view of the patient's age, physical condition, and tumour size, surgical resection was not performed.
  • 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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13. Nanba Y, Oka A, Ohno K: [Severe diarrhea associated with X-linked lissencephaly with absent corpus callosum and abnormal genitalia: a case report of successful treatment with the somatostatin analogue octreotide]. No To Hattatsu; 2007 Sep;39(5):379-82
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  • [Title] [Severe diarrhea associated with X-linked lissencephaly with absent corpus callosum and abnormal genitalia: a case report of successful treatment with the somatostatin analogue octreotide].
  • At the age of 2 years while being treated with the antiallergic formula, he was affected with severe diarrhea that resembled watery diarrhea-hypokalemia-acidosis syndrome (WDHA).
  • Hypoglycemia without hyperinsulinemia was seen during fasting, and plasma vasoactive intestinal polypeptide was not increased when he had WDHA-like diarrhea.
  • Although pancreas of ARX mutant mice revealed an increased number of beta and delta cells, we did not detect the cause of hypoglycemia and secretory diarrhea by pancreatic endocrinology.
  • His urinary findings mimicked the symptoms of Fanconi syndrome, so it was possible that his hyperaldsteronemia affected not only his intestinal tract, but also his renal tubules.
  • [MeSH-major] Abnormalities, Multiple / genetics. Agenesis of Corpus Callosum. Diarrhea / drug therapy. Gastrointestinal Agents / therapeutic use. Genes, X-Linked. Genitalia, Male / abnormalities. Octreotide / therapeutic use

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  • (PMID = 17879613.001).
  • [ISSN] 0029-0831
  • [Journal-full-title] No to hattatsu. Brain and development
  • [ISO-abbreviation] No To Hattatsu
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Gastrointestinal Agents; RWM8CCW8GP / Octreotide
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14. 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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  • [Title] Expression of somatostatin receptor and effects of somatostatin analog on pancreatic endocrine tumors.
  • PURPOSE: Somatostatin analogs have been administered to patients with pancreatic endocrine tumors in an attempt to inhibit hormone hypersecretion and prevent tumor growth.
  • The aim of this study was to immunohistochemically evaluate the expression of somatostatin receptor subtypes in human pancreatic endocrine tumors, and to determine whether the expression of these subtypes is correlated with the effectiveness of the somatostatin analogs.
  • 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.
  • RESULTS: Cells were homogeneously stained in the tumor region.
  • There was no difference among the immunohistochemical stainings of somatostatin receptor subtypes according to most tumor characteristics; however, the expression of sst 2 was extremely positive, and the expression of sst 3 was moderately positive in the specimen from a patient in whom the octreotide injection had proven very effective.
  • [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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15. 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.
  • A complete resection of the primary tumor was achieved in 82%, and 12 of 15 are alive, 10 with no evidence of disease.
  • 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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16. 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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  • [Title] Circulating ghrelin levels in patients with pancreatic and gastrointestinal neuroendocrine tumors: identification of one pancreatic ghrelinoma.
  • Although the expression of ghrelin has been demonstrated in most gastrointestinal carcinoids and pancreatic tumors, the circulating levels of this peptide have been marginally assessed in patients with these disorders.
  • 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.
  • In conclusion, the study showed that carcinoids and pancreatic tumors rarely cause ghrelin hypersecretion.
  • However, in this series, 1 pancreatic ghrelinoma not associated with clinical features of acromegaly was identified.
  • [MeSH-major] Carcinoma, Neuroendocrine / blood. Gastrointestinal Neoplasms / blood. Pancreatic Neoplasms / blood. Peptide Hormones / blood
  • [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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17. Melen-Mucha G, Lawnicka H, Kierszniewska-Stepien D, Komorowski J, Stepien H: The place of somatostatin analogs in the diagnosis and treatment of the neuoroendocrine glands tumors. Recent Pat Anticancer Drug Discov; 2006 Jun;1(2):237-54
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  • [Title] The place of somatostatin analogs in the diagnosis and treatment of the neuoroendocrine glands tumors.
  • The synthesis of the first two metabolically stabilized and more potent SS analogs, octreotide and lanreotide leads to the establishment of applications for them and to introduction into routine therapies.
  • Similarly, these drugs are also very effective in the treatment of TSH-secreting adenomas.
  • The introduction of these drugs into therapy of the functional neuroendocrine tumors of the gastrointestinal tract was a crucial step in the treatment.
  • Octreotide and lanreotide are the drugs of choice in the treatment of patients with: VIPoma, glucagonoma and carcinoid syndrome.
  • Somatostatin receptor scintigraphy with OctreoScan has been recommended as the best imaging technique in these tumors in the localization and staging procedure.
  • SS analogs, coupled to radioisotope or cytotoxic drugs, create another class of SS molecules, very promising in the therapy of the endocrine glands tumors and in other tumors.
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / drug therapy. Somatostatin / analogs & derivatives. Somatostatin / therapeutic use
  • [MeSH-minor] Animals. Humans. Patents as Topic. Positron-Emission Tomography. Receptors, Somatostatin / drug effects. Receptors, Somatostatin / metabolism

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  • (PMID = 18221040.001).
  • [ISSN] 1574-8928
  • [Journal-full-title] Recent patents on anti-cancer drug discovery
  • [ISO-abbreviation] Recent Pat Anticancer Drug Discov
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United Arab Emirates
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Receptors, Somatostatin; 51110-01-1 / Somatostatin
  • [Number-of-references] 190
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18. Dadan J, Wojskowicz P, Wojskowicz A: Neuroendocrine tumors of the pancreas. Wiad Lek; 2008;61(1-3):43-7
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  • [Title] Neuroendocrine tumors of the pancreas.
  • The neuroendocrine tumors (NET) of the pancreas are very rare lesions with frequency of about 3 to 10 per 1 000 000 inhabitants.
  • The neuroendocrine tumors composes a heterogeneous group of tumors.
  • The gastro-entero-pancreatic tumors (GEP) constitute 70% of all NET and 2% of all digestive system tumors.
  • There have been several attempts to classify those lesions and since 2000 exists WHO classification which divides NET according to malignancy and histologic structure.
  • The most often NET of the pancreas are insulinoma, gastrinoma, glucagonoma, somatostatinoma, VIPoma.
  • There is a recommendation to assay hormonal activity, measure concentration of specific peptides, biogenic amines and hormones produced by NET cells to establish diagnosis.
  • 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.
  • The neuroendocrine tumors should be treated in centers with highest rank of references.
  • [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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19. 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.
  • The patients presented with Verner-Morrison, glucagonoma, Zollinger Ellison and carcinoid syndrome respectively.
  • 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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20. Fernández-Martínez AB, Bajo AM, Valdehita A, Isabel Arenas M, Sánchez-Chapado M, Carmena MJ, Prieto JC: Multifunctional role of VIP in prostate cancer progression in a xenograft model: suppression by curcumin and COX-2 inhibitor NS-398. Peptides; 2009 Dec;30(12):2357-64
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  • [Title] Multifunctional role of VIP in prostate cancer progression in a xenograft model: suppression by curcumin and COX-2 inhibitor NS-398.
  • We used an in vivo model of human experimental prostate cancer in order to shed a new light on the effects of vasoactive intestinal peptide (VIP) on tumor growth as well as its pro-metastatic potential in this disease.
  • The regulatory role of VIP on cyclooxygenase-2 (COX-2) and vascular endothelial growth factor (VEGF) expression as well as on matrix metalloproteinase-2 and 9 (MMP-2 and 9) activities was examined.
  • A selective COX-2 inhibitor, NS-398, and curcumin were used to block VIP effects.
  • Xenografts of VIP-treated PC3 prostate cancer cells in nude mice gave tumors that grew significantly faster than those in the untreated group.
  • It is conceivably a result of both the trophic effect of VIP on prostate cancer cells and the proangiogenic action of the neuropeptide in the growing tumor.
  • We show the overexpression at mRNA and/or protein levels of VIP, its main receptor VPAC(1), the major angiogenic factor VEGF, and the pro-inflammatory enzyme COX-2 as well as the increased activity of MMP-2 and 9 in tumors derived from VIP-treated PC3 cells as compared with control group.
  • The overexpression of the above biomarkers was suppressed in tumors derived from VIP-treated PC3 cells that had been previously incubated with curcumin or NS-398.
  • Thus, the potential therapeutic role of curcumin and selective COX-2 inhibitors in combination with available VIP antagonists should be considered in prostate cancer therapy as supported by their inhibitory activities on tumor cell growth.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Curcumin / pharmacology. Nitrobenzenes / pharmacology. Prostatic Neoplasms / metabolism. Prostatic Neoplasms / pathology. Sulfonamides / pharmacology. Vasoactive Intestinal Peptide / physiology
  • [MeSH-minor] Animals. Cell Line, Tumor. Cell Proliferation / drug effects. Cyclooxygenase 2. Humans. Immunohistochemistry. Male. Matrix Metalloproteinase 2 / metabolism. Mice. Mice, Nude. Polymerase Chain Reaction. Xenograft Model Antitumor Assays

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  • (PMID = 19772879.001).
  • [ISSN] 1873-5169
  • [Journal-full-title] Peptides
  • [ISO-abbreviation] Peptides
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Nitrobenzenes; 0 / Sulfonamides; 123653-11-2 / N-(2-cyclohexyloxy-4-nitrophenyl)methanesulfonamide; 37221-79-7 / Vasoactive Intestinal Peptide; EC 1.14.99.- / Ptgs2 protein, mouse; EC 1.14.99.1 / Cyclooxygenase 2; EC 3.4.24.24 / Matrix Metalloproteinase 2; IT942ZTH98 / Curcumin
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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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