VIPoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
A VIPoma (also known as Verner Morrison syndrome, after the physicians who first described it [1]) is a rare (1 per 10,000,000 per year) endocrine tumor, usually (about 90%) originating in the pancreas, which produces vasoactive intestinal peptide (VIP).
It is a syndrome caused by non-β islet-cell tumors. It may be associated with multiple endocrine neoplasia.
The massive amounts of VIP in turn cause profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria (hence WDHA-syndrome, or pancreatic cholera syndrome), acidosis, vasodilation (flushing and hypotension), hypercalcemia and hyperglycemia.[2]
Historical Perspective
VIPoma was first described in 1958 by Verner and Morrison.[3]
Pathophysiology
A VIPoma is a rare tumor of the non-beta cells of the pancreas that results in the overproduction of the hormone vasoactive intestinal peptide (VIP). On microscopic histopathological analysis, findings of VIPoma are composition of uniform, small to intermediate-sized cells in clusters, nests, and trabecular growth patterns with hyperchromatic nuclei and scant cytoplasm.[4][5]
Causes
There are no established causes for VIPoma.[6]
Differential Diagnosis
VIPoma must be differentiated from ganglioneuroblastoma, ganglioneuroma, factitious diarrhea, bilt salt enteropathy, rectal vilous adenomas, and laxative abuse.[7][8]
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
History and Symptoms
Physical Examination
Laboratory Findings
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
References
- ↑ Verner, J. V., and Morrison, A. B. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med 1958; 374: 1958.
- ↑ Mansour JC, Chen H. Pancreatic endocrine tumors. J Surg Res 2004; 120: 139-61. PMID 15172200
- ↑ Maheshwari RR, Desai M, Rao VP, Palanki RR, Namburi RP, Reddy KT; et al. (2013). "Ischemic stroke as a presenting feature of VIPoma due to MEN 1 syndrome". Indian J Endocrinol Metab. 17 (Suppl 1): S215–8. doi:10.4103/2230-8210.119576. PMC 3830309. PMID 24251163.
- ↑ Natanzi, Naveed; Amini, Mazyar; Yamini, David; Nielsen, Shawn; Ram, Ramin (2009). "Vasoactive Intestinal Peptide Tumor". Scholarly Research Exchange. 2009: 1–7. doi:10.3814/2009/938325. ISSN 1687-8299.
- ↑ Joyce, David L; Hong, Kelvin; Fishman, Elliot K; Wisell, Joshua; Pawlik, Timothy M (2008). "Multi-visceral resection of pancreatic VIPoma in a patient with sinistral portal hypertension". World Journal of Surgical Oncology. 6 (1): 80. doi:10.1186/1477-7819-6-80. ISSN 1477-7819.
- ↑ VIPoma. U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000228.htm. Accessed on October 19, 2015
- ↑ Reindl T, Degenhardt P, Luck W, Riebel T, Sarioglu N, Henze G; et al. (2004). "[The VIP-secreting tumor as a differential diagnosis of protracted diarrhea in pediatrics]". Klin Padiatr. 216 (5): 264–9. doi:10.1055/s-2004-44901. PMID 15455292.
- ↑ Elshafie O, Grant C, Al-Hamdani A, Jain R, Woodhouse N (2011). "VIPoma Crisis: Immediate and life saving reduction of massive stool volumes on starting treatment with octreotide". Sultan Qaboos Univ Med J. 11 (1): 104–7. PMC 3074686. PMID 21509215.