Abstract
Background. Although pancreatic endocrine tumor can produce a variety of hormones, few pancreatic tumors produce a high systemic calcitonin concentration. Furthermore, calcitonin-producing pancreatic tumors rarely produce elevations of VIP in addition.
Methods. We evaluated and treated a 50-yr-old woman with the WDHA syndrome. Abdominal computed tomography (CT) detected a tumor in the tail of the pancreas. Peripheral plasma calcitonin and VIP concentrations were markedly increased to 2000 pg/mL (normal, <74 pg/mL) and 7200 pg/mL (normal, <100 pg/mL), respectively. We diagnosed a calcitonin- and VIP-producing pancreatic endocrine tumor, which was removed by distal pancreatectomy including splenectomy.
Results. Plasma calcitonin and VIP were determined in blood from the vein draining the tumor and splenic vein, sampled at operation. These secreted concentrations were extremely high: 4640 and 3610 pg/mL for calcitonin; 24700 and 13500 pg/mL for VIP. Calcitonin and VIP were also highly elevated in the resected tumor. Plasma calcitonin and VIP rapidly decreased after tumor resection. The patient has been well without recurrence for over 20 yr.
Conclusion. An unusual pancreatic tumor secreting vasoactive intestinal peptide (VIP) caused WDHA syndrome (watery diarrhea, hypokalemia, and achlorhydria/hypochlorhydria) and also hypercalcemia. The latter was only partially offset by a large excess of calcitonin also secreted by the tumor.
Similar content being viewed by others
References
Fleury A, Flejou JF, Sauvanet A, et al. Calcitonin-secreting tumors of the pancreas: about six cases. Pancreas 1998;16:545–550.
Soga J, Yakuwa Y. Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J Exp Clin Canc Res 1998;17:389–400.
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;124:1050–1055.
Permert J, Mogaki M, Andren-Sandberg A, et al. Pancreatic mixed ductal-islet tumors. Is this an entity? Int J Pancreatol 1992;11:23–29.
Eriksson B, Oberg K, Andersson T, et al. Treatment of malignant endocrine pancreatic tumors with a new longacting somatostatin analogue, SMS 20-995. Scand J Gastroenterol 1988;23:508–512.
Oberg K, Bostrom H, Fahrenkrug J, et al. Streptozotocin treatment of a pancreatic tumor producing VIP and gastrin associated with Verner-Morrison syndrome. Acta Med Scand 1979;206:223–227.
Burbige EJ, Manning RJ, Belber JP. Watery diarrhea syndrome with elevated levels of vasoactive intestinal polypeptide associated with pancreatitis and pancreatic pseudocyst. Am J Gastroenterol 1978;70:136–140.
Rambaud JC, Galian A, Scotto J, et al. Pancreatic cholera (W.D.H.A. syndrome). Histochemical and ultrastructural studies. Virch Arch A, Pathol Anat Histol 1975;367:35–45.
Rambaud JC, Hautefeuille M, Ruskone A, Jacquenod P. Diarrhoea due to circulating agents. Clin Gastroenterol 1986;15:603–629.
Gutniak M, Rosenqvist U, Grimelius L, et al. Report on a patient with watery diarrhoea syndrome caused by a pancreatic tumour containing neurotensin, enkephalin and calcitonin. Acta Med Scand 1980;208:95–100.
Oberg K, Loof L, Bostrom H, et al. Hypersecretion of calcitonin in patients with the Verner-Morrison syndrome. Scand J Gastroenterol 1981;16:135–144.
Rambaud JC, Jian R, Flouri B, et al. Pathophysiological study of diarrhoea in a patient with medullary thyroid carcinoma. Evidence against a secretory mechanism and for the role of shortened transit time. Gut 1988;29:537–543.
Findlay DM, Martin TJ. Receptors of calciotropic hormones. Horm Metab Res 1997;29:128–134.
Hohmann EL, Levine L, Tashjian AH. Vasoactive intestinal peptide stimulates bone resorption via a cyclic adenosine 3′, -5′-morphosphate-dependent mechanism. Endocrinology 1983;112:1233–1239.
Mao C, Carter P, Schaefer P, et al. Malignantislet cell tumor associated with hypercalcemia. Surgery 1995;117:37–40.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ichimura, T., Kondo, S., Okushiba, S. et al. A Calcitonin and vasoactive intestinal peptide-producing pancreatic endocrine tumor associated with the WDHA syndrome. Int J Gastrointest Canc 33, 99–102 (2003). https://doi.org/10.1385/IJGC:33:2-3:99
Issue Date:
DOI: https://doi.org/10.1385/IJGC:33:2-3:99