The major clinical features are prolonged watery diarrhea (fasting stool volume > 750 to 1000 mL/day) and symptoms of hypokalemia and dehydration. Half of the patients have relatively constant diarrhea while the rest have alternating periods of severe and moderate diarrhea. One third have diarrhea < 1yr before diagnosis, but in 25%, diarrhea is present for 5 yr or more before diagnosis. Lethargy, muscle weakness, nausea, vomiting and crampy abdominal pain are frequent symptoms. Hypokalemia and impaired glucose tolerance occur in < 50% of patients. Achlorhydria is also a feature. During attacks of diarrhea, flushing similar to the carcinoid syndrome occur rarely.[5]
Blood chemistry tests (basic or comprehensive metabolic panel)
CT scan of the abdomen
MRI of the abdomen
Stool examination for the cause of diarrhea and electrolyte levels
Vasoactive intestinal peptide (VIP) level in the blood[6]
Treatmentedit
The first goal of treatment is to correct dehydration. Fluids are often given through a vein (intravenous fluids) to replace fluids lost in diarrhea. The next goal is to slow the diarrhea. Some medications can help control diarrhea. Octreotide, which is a human-made form of the natural hormone somatostatin, blocks the action of VIP.[citation needed]
The best chance for a cure is surgery to remove the tumor. If the tumor has not spread to other organs, surgery can often cure the condition.[citation needed]
For metastatic disease, peptide receptor radionuclide therapy (PRRT) can be highly effective. This treatment involves attaching a radionuclide (Lutetium-177 or Yttrium-90) to a somatostatin analogue (octreotate or octreotide). This is a novel way to deliver high doses of beta radiation to kill tumours. Some people seem to respond to a combination chemo called capecitabine and temozolomide but there is no report that it totally cured people of VIPoma.[citation needed]
Prognosisedit
Surgery can usually cure VIPomas. However, in one-third to one-half of patients, the tumor has spread by the time of diagnosis and cannot be cured.[citation needed]
^"VIPoma | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2018-04-17.
^Verner JV, Morrison AB (Sep 1958). "Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia". Am J Med. 25 (3): 374–80. doi:10.1016/0002-9343(58)90075-5. PMID 13571250.
^"Carcinoid Tumors and Syndrome". The Lecturio Medical Concept Library. Retrieved 5 July 2021.
^ abSandhu S, Jialal I. "ViPoma". National Center for Biotechnology Information, U.S. National Library of Medicine. Retrieved 5 July 2021.
Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease . 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 32.
National Cancer Institute. Islet cell tumors (pancreatic) treatment PDQ. Updated October 31, 2008.
External linksedit
May 08, 2024
vipoma, vipoma, rare, endocrine, tumor, that, overproduces, vasoactive, intestinal, peptide, thus, incidence, about, year, usually, about, originate, from, islet, cells, pancreas, they, sometimes, associated, with, multiple, endocrine, neoplasia, type, roughly. A VIPoma or vipoma v ɪ ˈ p oʊ m e is a rare endocrine tumor 1 that overproduces vasoactive intestinal peptide thus VIP oma The incidence is about 1 per 10 000 000 per year VIPomas usually about 90 originate from the non b islet cells of the pancreas They are sometimes associated with multiple endocrine neoplasia type 1 Roughly 50 75 of VIPomas are malignant but even when they are benign they are problematic because they tend to cause a specific syndrome the massive amounts of VIP cause a syndrome of profound and chronic watery diarrhea and resultant dehydration hypokalemia achlorhydria acidosis flushing and hypotension from vasodilation hypercalcemia and hyperglycemia 2 3 This syndrome is called Verner Morrison syndrome VMS WDHA syndrome from watery diarrhea hypokalemia achlorhydria or pancreatic cholera syndrome PCS The eponym reflects the physicians who first described the syndrome 4 VIPomaSpecialtyOncology Contents 1 Symptoms and signs 2 Diagnosis 3 Treatment 4 Prognosis 5 References 6 External linksSymptoms and signs editThe major clinical features are prolonged watery diarrhea fasting stool volume gt 750 to 1000 mL day and symptoms of hypokalemia and dehydration Half of the patients have relatively constant diarrhea while the rest have alternating periods of severe and moderate diarrhea One third have diarrhea lt 1yr before diagnosis but in 25 diarrhea is present for 5 yr or more before diagnosis Lethargy muscle weakness nausea vomiting and crampy abdominal pain are frequent symptoms Hypokalemia and impaired glucose tolerance occur in lt 50 of patients Achlorhydria is also a feature During attacks of diarrhea flushing similar to the carcinoid syndrome occur rarely 5 Diagnosis editBesides the clinical picture fasting VIP plasma level may confirm the diagnosis and CT scan and somatostatin receptor scintigraphy are used to localise the tumor which is usually metastatic at presentation 6 Tests include Blood chemistry tests basic or comprehensive metabolic panel CT scan of the abdomen MRI of the abdomen Stool examination for the cause of diarrhea and electrolyte levels Vasoactive intestinal peptide VIP level in the blood 6 Treatment editThe first goal of treatment is to correct dehydration Fluids are often given through a vein intravenous fluids to replace fluids lost in diarrhea The next goal is to slow the diarrhea Some medications can help control diarrhea Octreotide which is a human made form of the natural hormone somatostatin blocks the action of VIP citation needed The best chance for a cure is surgery to remove the tumor If the tumor has not spread to other organs surgery can often cure the condition citation needed For metastatic disease peptide receptor radionuclide therapy PRRT can be highly effective This treatment involves attaching a radionuclide Lutetium 177 or Yttrium 90 to a somatostatin analogue octreotate or octreotide This is a novel way to deliver high doses of beta radiation to kill tumours Some people seem to respond to a combination chemo called capecitabine and temozolomide but there is no report that it totally cured people of VIPoma citation needed Prognosis editSurgery can usually cure VIPomas However in one third to one half of patients the tumor has spread by the time of diagnosis and cannot be cured citation needed References edit VIPoma at Dorland s Medical Dictionary Mansour JC Chen H Jul 2004 Pancreatic endocrine tumors J Surg Res 120 1 139 61 doi 10 1016 j jss 2003 12 007 PMID 15172200 VIPoma Genetic and Rare Diseases Information Center GARD an NCATS Program rarediseases info nih gov Retrieved 2018 04 17 Verner JV Morrison AB Sep 1958 Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia Am J Med 25 3 374 80 doi 10 1016 0002 9343 58 90075 5 PMID 13571250 Carcinoid Tumors and Syndrome The Lecturio Medical Concept Library Retrieved 5 July 2021 a b Sandhu S Jialal I ViPoma National Center for Biotechnology Information U S National Library of Medicine Retrieved 5 July 2021 Jensen RT Norton JA Endocrine tumors of the pancreas and gastrointestinal tract In Feldman M Friedman LS Brandt LJ eds Sleisenger and Fordtran s Gastrointestinal and Liver Disease 9th ed Philadelphia Pa Saunders Elsevier 2010 chap 32 National Cancer Institute Islet cell tumors pancreatic treatment PDQ Updated October 31 2008 External links edit Retrieved from https en wikipedia org w index php title VIPoma amp oldid 1086879401, wikipedia, wiki, book, books, library,
