Summary
A case in which a pheochromocytoma secreted vasoactive intestinal peptide, causing WDHA syndrome, is reported. The patient, a 43-year-old woman, was seen because of intractable watery diarrhea, hypokalemia and weight loss. She was found to have a mass in the right adrenal area. Preoperatively, vasoactive intestinal peptide levels were elevated, and the diagnosis of WDHA syndrome was entertained. Exploratory laparotomy revealed a tumor of the right adrenal gland, measuring 15×15 cm, which was resected. Histologic examination revealed it to be a pheochromocytoma. Postoperatively, vasoactive intestinal peptide returned to normal. The patient had complete remission of symptoms, and has remained well since.
Similar content being viewed by others
References
Barbezat GO, Grossman MI: Cholera-like diarrhoea induced by glucagon plus gastrin. Lancet 1: 1025, 1971
Bloom SR, Polak JM, Pearse AG: Vasoactive intestinal peptide and watery-diarrhoea syndrome. Lancet 2: 14, 1973
Bodanszky M, Klausner YS, Said SI: Biological activities of synthetic peptides corresponding to fragments of and to the entire sequence of the vasoactive intestinal peptide (dog/solution synthesis/azide coupling). Proc Natl Acad Sci USA 70: 382, 1973
Brown CH, Crile G Jr: Pancreatic adenoma with intractable diarrhea, hypokalemia and hypercalcemia. JAMA 190: 30, 1964
Chears WC Jr, Thompson JE, Hutcheson JB, et al: Pancreatic islet tumor with severe diarrhea. Am J Med 29: 529, 1960
Elias E, Polak JM, Bloom SR, et al: Pancreatic cholera due to prodduction of gastric inhibitory polypeptide. Lancet 2: 791, 1972
Fausa O, Fretheim B, Eljon K, et al: Intractable watery diarrhoea, hypokalaemia—achlorhydria associated with non-pancreatic retroperitoneal neurogenous tumour-containing vasoactive intestinal peptide. Scand J Gastroenterol 8: 713, 1973
Hindle W, McBrien DJ, Creamer B: Watery diarrhoea and an islet cell tumour Gut 5: 359, 1964
Jacobs WH, Halperin P, Mantz FA: Watery diarrhea and hypokalemia due to nonbeta-islet cell hyperplasia of the pancreas. Am J Gastroenterol 57: 333, 1972
Kerins C, Said SI: Hyperglycemic and glycogenolytic effects of vasoactive intestinal polypeptide. Proc Soc Exp Biol Med
Kraft AR, Tompkins RK, Zollinger RM: Recognition and management of the diarrheal syndrome caused by non-beta islet-cell tumors of the pancreas. Am J Surg 119: 163, 1970
Loehry CA, Kingham JG, Whorwell PJ: Watery diarrhoea, hypokalaemia associated with a phaeochromocytoma. Postgrad Med J 51: 416, 1975
Lopes VM, Reis DD, Cunha AB: Islet-cell adenoma of the pancreas with reversible watery diarrhea and hypokalemia. Am J Gastroenterol 53: 17, 1970
Marks IN, Bank S, Louw JH: Islet-cell tumor of the pancreas with reversible watery diarrhea and achlorhydria. Gastroenterology 52: 695, 1967
Matsumoto KK, Peter JB, Raymond G, et al: Watery diarrhea and hypokalemia associated with pancreatic islet-cell adenoma. Gastroenterology 50: 231, 1966
Murray JS, Paton RR, Pope CE II: Pancreatic tumor associated with flushing and diarrhea: Report of a case. N Engl J Med 264: 436, 1961
Parkins RA: Severe watery diarrhoea and potassium depletion associated with an islet-cell tumour of pancreas. Br Med J 2: 356, 1961
Pearse AG, Polak JM: Endocrine tumours of neural crest origin: Neurolophomas, apudomas and the apud concept. Med Biol 52: 3, 1974
Priest WM, Alexander MK: Islet-cell tumour of the pancreas with peptide ulceration, diarrhoea, and hypokalaemia. Lancet 2: 1145, 1957
Rambaud JC, Modigliani R, Matuchansky C, et al: Pancreatic cholera: Studies on tumoral secretions and pathophysiology of diarrhea. Gastroenterology 69: 110, 1975
Said SI: Vasoactive intestinal polypeptide: Elevated plasma and tissue levels in the watery-diarrhea syndrome due to pancreatic and other tumors. Trans Assoc Am Physicians 88: 87, 1975
Said SI, Faloona GR: Elevated plasma and tissue levels of vasoactive intestinal polypeptide in the watery-diarrhea syndrome due to pancreatic, bronchogenic and other tumors. N Engl J Med 293: 155, 1975
Said SI, Mutt V: Polypeptide with broad biological activity: Isolation from small intestine. Science 169: 1217, 1970
Schorr BA, Said SI, Makhlouf GM: Inhibition of gastric secretion by synthetic vasointestinal peptide (VIP). Clin Res 22: 23A, 1974
Schwartz CJ, Kimberg DV, Sheerin HE, et al: Vasoactive intestinal peptide stimulation of adenylate cyclase and active electrolyte secretion in intestinal mucosa. J Clin Invest 54: 536, 1974
Seijffers MJ, Micha L, Hermann G: Intractable watery diarrhea, hypokalemia, and malabsorption in a patient with mediterranean type of abdominal lymphoma. Gastroenterology 55: 118, 1968
Stoker DJ, Wynn V: Pancreatic islet-cell tumour with watery diarrhoea and hypokalaemia. Gut 11: 911, 1970
Swift PG, Bloom SR, Harris F. Watery diarrhoea and ganglioneuroma with secretion of vasoactive intestinal peptide. Arch Dis Child 50: 896, 1975
Telling M, Smiddy FG: Islet tumours of the pancreas with intractable diarrhoea, Gut 2: 12, 1961
Udall JN, Singer DB, Huang CT, et al: Watery diarrhea and hypokalemia associated with increased plasma vasoactive intestinal peptide in a child. J Pediatr 88: 819, 1976
Verner JV, Morrison AB: Islet-cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med 25: 374, 1958
Verner JV, Morrison AB: Non-b islet tumours and the syndrome of watery diarrhoea, hypokalemia and hypochlorhydria, Clin Gastroenterol 3: 595 (Sept) 1974
Author information
Authors and Affiliations
Additional information
Division of General Surgery.
About this article
Cite this article
Matta, M.K., Prorok, J.J., Trimpi, H.D. et al. WDHA syndrome caused by pheochromocytoma: Report of a case. Dis Colon Rectum 21, 297–301 (1978). https://doi.org/10.1007/BF02586708
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF02586708