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
VIPoma was first described in 1958 by Verner and Morrison.[1] 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.[2][3] There are no established causes for VIPoma.[4] VIPoma must be differentiated from ganglioneuroblastoma, ganglioneuroma, factitious diarrhea, bile salt enteropathy, rectal vilous adenomas, and laxative abuse.[5][6] The incidence VIPoma is approximately 0.01 per 100,000 individuals worldwide. Females are more commonly affected with VIPoma than male. The incidence of VIPoma increases with age, the median age at diagnosis is 50 years.[7] The most common risk factor in the development of VIPoma is positive family history of multiple endocrine neoplasia type 1.[8] If left untreated, patients with VIPoma may progress to develop watery diarrhea, abdominal pain, bloating, nausea, vomiting, skin rash, backache, flushing, and lethargy.[2] Common complications of VIPoma include metastasis, cardiac arrest from low blood potassium level, and dehydration. The presence of metastasis is associated with a particularly poor prognosis among patients with VIPoma, with a 5 year survival rate of 20% and 3 year survival rate of 40%.[9] The hallmark of VIPoma is watery diarrhea. A positive history of abdominal pain, weight loss, numbness, and weakness is suggestive of VIPoma.[10][11][12][13] Common physical examination findings of VIPoma include tachycardia, rash, facial flushing, abdominal tenderness, and abdominal distention. Laboratory tests used in the diagnosis of VIPoma include serum vasoactive intestinal polypeptide (VIP) levels, basal gastric acid output, potassium, bicarbonate, magnesium, and calcium levels.[12][13] On CT scan VIPoma is characterized by hypervascularity with diffuse multiple metastatic nodulation.[14] Abdominal MRI is helpful in the diagnosis of VIPoma. On abdominal MRI, VIPoma is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. Abdominal ultrasound scan may be helpful in the diagnosis of VIPoma. Finding on ultrasound scan suggestive of VIPoma is hypoechoic tumor in the distal pancreas.[14] Other imaging studies for VIPoma include somatostatin receptor scintigraphy and PET scan. Initial treatment in patient with VIPoma is prompt replacement of fluid and electrolyte losses. Steroids may be used to provide symptomatic relief.[15] Surgery is the mainstay of treatment for VIPoma.[16] Secondary prevention measures of VIPoma include a detailed history, physical examination, and imaging every 3 to 12 months up to one year post resection and every 6 to 12 months thereafter.[15]
Historical Perspective
VIPoma was first described in 1958 by Verner and Morrison.[1]
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.[2][3]
Causes
There are no established causes for VIPoma.[4]
Differentiating VIPoma From Other Diseases
VIPoma must be differentiated from ganglioneuroblastoma, ganglioneuroma, factitious diarrhea, bile salt enteropathy, rectal vilous adenomas, and laxative abuse.[5][6]
Epidemiology and Demographics
The incidence VIPoma is approximately 0.01 per 100,000 individuals worldwide. Females are more commonly affected with VIPoma than male. The incidence of VIPoma increases with age, the median age at diagnosis is 50 years.[7]
Risk Factors
The most common risk factor in the development of VIPoma is positive family history of multiple endocrine neoplasia type 1.[8]
Screening
According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for VIPoma.[17]
Natural History, Complications and Prognosis
If left untreated, patients with VIPoma may progress to develop watery diarrhea, abdominal pain, bloating, nausea, vomiting, skin rash, backache, flushing, and lethargy.[2] Common complications of VIPoma include metastasis, cardiac arrest from low blood potassium level, and dehydration. The presence of metastasis is associated with a particularly poor prognosis among patients with VIPoma, with a 5 year survival rate of 20% and 3 year survival rate of 40%.[9]
History and Symptoms
The hallmark of VIPoma is watery diarrhea. A positive history of abdominal pain, weight loss, numbness, and weakness is suggestive of VIPoma.[10][11][12][13]
Physical Examination
Common physical examination findings of VIPoma include tachycardia, rash, facial flushing, abdominal tenderness, and abdominal distention.
Laboratory Findings
Laboratory tests used in the diagnosis of VIPoma include serum vasoactive intestinal polypeptide (VIP) levels, basal gastric acid output, potassium, bicarbonate, magnesium, and calcium levels.[12][13]
CT
On CT scan VIPoma is characterized by hypervascularity with diffuse multiple metastatic nodulation.[14]
MRI
Abdominal MRI is helpful in the diagnosis of VIPoma. On abdominal MRI, VIPoma is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI.
Echocardiography or Ultrasound
Abdominal ultrasound scan may be helpful in the diagnosis of VIPoma. Finding on ultrasound scan suggestive of VIPoma is hypoechoic tumor in the distal pancreas.[14]
Other Imaging Findings
Other imaging studies for VIPoma include somatostatin receptor scintigraphy and PET scan.
Medical Therapy
Initial treatment in patient with VIPoma is prompt replacement of fluid and electrolyte losses. Steroids may be used to provide symptomatic relief.[15]
Surgery
Surgery is the mainstay of treatment for VIPoma.[16]
Primary Prevention
There is no established method for prevention of VIPoma.
Secondary Prevention
Secondary prevention measures of VIPoma include a detailed history, physical examination, and imaging every 3 to 12 months up to one year post resection and every 6 to 12 months thereafter.[15]
References
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 2.2 2.3 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.
- ↑ 3.0 3.1 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.
- ↑ 4.0 4.1 VIPoma. U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000228.htm. Accessed on October 19, 2015
- ↑ 5.0 5.1 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.
- ↑ 6.0 6.1 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.
- ↑ 7.0 7.1 Joyce DL, Hong K, Fishman EK, Wisell J, Pawlik TM (2008). "Multi-visceral resection of pancreatic VIPoma in a patient with sinistral portal hypertension". World J Surg Oncol. 6: 80. doi:10.1186/1477-7819-6-80. PMC 2517072. PMID 18662399.
- ↑ 8.0 8.1 VIPoma. Known Cancer. http://www.knowcancer.com/oncology/vipoma/. Accessed on October 19, 2015.
- ↑ 9.0 9.1 Smith, Stephen L.; Branton, Susan A.; Avino, Anthony J.; Martin, J.Kirk; Klingler, Paul J.; Thompson, Geoffrey B.; Grant, Clive S.; van Heerden, Jon A. (1998). "Vasoactive intestinal polypeptide secreting islet cell tumors: A 15-year experience and review of the literature". Surgery. 124 (6): 1050–1055. doi:10.1067/msy.1998.92005. ISSN 0039-6060.
- ↑ 10.0 10.1 VIPoma. U.S. National Library of Medicine. Accessed on October 23, 2015. https://www.nlm.nih.gov/medlineplus/ency/article/000228.htm
- ↑ 11.0 11.1 VIPoma. Wikipedia. Accessed on October 23, 2015. https://en.wikipedia.org/wiki/VIPoma
- ↑ 12.0 12.1 12.2 12.3 Remme CA, de Groot GH, Schrijver G (2006). "Diagnosis and treatment of VIPoma in a female patient". Eur J Gastroenterol Hepatol. 18 (1): 93–9. PMID 16357627.
- ↑ 13.0 13.1 13.2 13.3 Ghaferi AA, Chojnacki KA, Long WD, Cameron JL, Yeo CJ (2008). "Pancreatic VIPomas: subject review and one institutional experience". J Gastrointest Surg. 12 (2): 382–93. doi:10.1007/s11605-007-0177-0. PMID 17510774.
- ↑ 14.0 14.1 14.2 14.3 Apodaca-Torrez FR, Triviño M, Lobo EJ, Goldenberg A, Triviño T (2014). "Extra-pancreatic vipoma". Arq Bras Cir Dig. 27 (3): 222–3. PMID 25184777.
- ↑ 15.0 15.1 15.2 15.3 Vinik A. Vasoactive Intestinal Peptide Tumor (VIPoma) [Updated 2013 Nov 28]. In: De Groot LJ, Beck-Peccoz P, Chrousos G, et al., editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000-. Available from: http://www.ncbi.nlm.nih.gov/books/NBK278960/
- ↑ 16.0 16.1 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors). National Cancer Institute. http://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq#section/_78. Accessed on October 21, 2015.
- ↑ http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=VIPoma. Accessed on October 19, 2015.