The VIPoma syndrome

INTRODUCTION

VIPomas are rare neuroendocrine tumors that secrete vasoactive intestinal polypeptide (VIP). They are detected in 1 in 10 million people per year [1]. The majority of VIPomas arise within the pancreas, and are classified as a pancreatic neuroendocrine (islet cell) tumor along with insulinoma, glucagonoma, somatostatinoma, and gastrinoma. However, other VIP-secreting tumors have been reported, including bronchogenic carcinoma, colon carcinoma, ganglioneuroblastoma, pheochromocytoma, hepatoma, and adrenal tumors. In children, VIPomas occur in sympathetic ganglia and in the adrenal glands [1]. (See "Classification, epidemiology, clinical presentation, localization, and staging of pancreatic neuroendocrine tumors (islet-cell tumors)", section on 'Classification and nomenclature'.)

Symptomatic pancreatic VIPomas are usually solitary, more than 3 cm in diameter, and occur in the tail of pancreas in 75 percent of patients. Approximately 60 to 80 percent of VIPomas have metastasized by the time of diagnosis [2,3].

VIPomas usually occur as isolated tumors, but are part of the multiple endocrine neoplasia syndrome type 1 (MEN1) in 5 percent of patients. The latter patients may also have primary hyperparathyroidism, pituitary tumors, gastrinoma, and other tumors. (See "Multiple endocrine neoplasia type 1: Clinical manifestations and diagnosis".) In one report of 580 patients with the MEN-1 syndrome, only 2 (0.65 percent) harbored a VIPoma [4].

PATHOPHYSIOLOGY

Vasoactive intestinal polypeptide (VIP)is a 28 amino acid polypeptide that binds to high affinity receptors on intestinal epithelial cells, leading to activation of cellular adenylate cyclase and cAMP production. This results in net fluid and electrolyte secretion into the lumen [5,6]. VIP also has other actions that may be clinically important in patients with a VIPoma (table 1). (See "Vasoactive intestinal polypeptide".)

The VIPoma syndrome is caused by excessive, unregulated secretion of VIP by the tumor. However, other substances, such as prostaglandin E2, may occasionally be secreted by the tumors [5].

                 

Subscribers log in here

To continue reading this article you must have access through your hospital or your group practice, log in to your personal subscription, or purchase a personal subscription. For more information, click below.
Literature review current through: Apr 2013. | This topic last updated: Aug 9, 2012.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2013 UpToDate, Inc.
References
Top
  1. Friesen SR. Update on the diagnosis and treatment of rare neuroendocrine tumors. Surg Clin North Am 1987; 67:379.
  2. Perry RR, Vinik AI. Clinical review 72: diagnosis and management of functioning islet cell tumors. J Clin Endocrinol Metab 1995; 80:2273.
  3. Smith SL, Branton SA, Avino AJ, et al. Vasoactive intestinal polypeptide secreting islet cell tumors: a 15-year experience and review of the literature. Surgery 1998; 124:1050.
  4. Lecorguillé M, Hammel P, Couvelard A, et al. [Jejunal vipoma]. Gastroenterol Clin Biol 2004; 28:797.
  5. Meriney DK. Pathophysiology and management of VIPoma: a case study. Oncol Nurs Forum 1996; 23:941.
  6. Bloom SR, Yiangou Y, Polak JM. Vasoactive intestinal peptide secreting tumors. Pathophysiological and clinical correlations. Ann N Y Acad Sci 1988; 527:518.
  7. Krejs GJ. VIPoma syndrome. Am J Med 1987; 82:37.
  8. Grier JF. WDHA (watery diarrhea, hypokalemia, achlorhydria) syndrome: clinical features, diagnosis, and treatment. South Med J 1995; 88:22.
  9. Mekhjian HS, O'Dorisio TM. VIPoma syndrome. Semin Oncol 1987; 14:282.
  10. Donowitz M, Kokke FT, Saidi R. Evaluation of patients with chronic diarrhea. N Engl J Med 1995; 332:725.
  11. American Gastroenterological Association medical position statement: guidelines for the evaluation and management of chronic diarrhea. Gastroenterology 1999; 116:1461.
  12. Kirkwood KS, Debas HT. Neuroendocrine tumors: common presentations of uncommon diseases. Compr Ther 1995; 21:719.
  13. Nikou GC, Toubanakis C, Nikolaou P, et al. VIPomas: an update in diagnosis and management in a series of 11 patients. Hepatogastroenterology 2005; 52:1259.
  14. AJCC (American Joint Committee on Cancer) Cancer Staging Manual, 7th ed, Edge SB, Byrd DR, Compton CC, et al (Eds), Springer, New York Vol 2010, p.241.
  15. Bilimoria KY, Bentrem DJ, Merkow RP, et al. Application of the pancreatic adenocarcinoma staging system to pancreatic neuroendocrine tumors. J Am Coll Surg 2007; 205:558.
  16. Eriksson B, Oberg K. An update of the medical treatment of malignant endocrine pancreatic tumors. Acta Oncol 1993; 32:203.
  17. O'Dorisio TM, Mekhjian HS, Gaginella TS. Medical therapy of VIPomas. Endocrinol Metab Clin North Am 1989; 18:545.
  18. Kraenzlin ME, Ch'ng JL, Wood SM, et al. Long-term treatment of a VIPoma with somatostatin analogue resulting in remission of symptoms and possible shrinkage of metastases. Gastroenterology 1985; 88:185.
  19. Lamberts SW, van der Lely AJ, de Herder WW, Hofland LJ. Octreotide. N Engl J Med 1996; 334:246.
  20. Newman CB, Melmed S, Snyder PJ, et al. Safety and efficacy of long-term octreotide therapy of acromegaly: results of a multicenter trial in 103 patients--a clinical research center study. J Clin Endocrinol Metab 1995; 80:2768.
  21. Moug SJ, Leen E, Horgan PG, Imrie CW. Radiofrequency ablation has a valuable therapeutic role in metastatic VIPoma. Pancreatology 2006; 6:155.
  22. National Comprehensive Cancer Network (NCCN) guidelines. Available at: www.nccn.org (Accessed on May 15, 2012).