A 45-Year-Old Female with Hypokalemic Rhabdomyolysis due to VIP-Producing Composite Pheochromocytoma

Abstract

The watery diarrhea, hypokalemia and achlorhydria (WHDA) syndrome due to vasoactive intestinal polypeptide (VIP)-producing extra-pancreatic tumors is rare. We report on a 45-year-old woman who suffered from persistent secretory diarrhea for six years and who was admitted to hospital with complaints of muscular weakness and myalgia. Biochemical testing revealed pronounced rhabdomyolysis due to severe hypokalemia. Gastrointestinal evaluation of long-standing diarrhea including endoscopy of the upper and lower gastrointestinal tract and the small intestine did not show any pathologies. An abdominal computed tomography scan revealed a mass of 4 × 5 cm in the left adrenal gland demonstrating a strong uptake in the 123I-labelled metaiodobenzylguanidine scintigraphy. Plasma levels of chromogranin A, calcitonin, parathormone, basal renin and most prominently VIP were increased in line with a increased 24 hour urinary secretion of noradrenaline, dopamine, normetanephrine and vanillymandelic acid. A WDHA (watery diarrhea, hypokalaemia, achlorhydria) syndrome with hypokalemic rhabdomyolysis due to a VIP-producing adrenal tumor was diagnosed that was removed surgically. The histological evaluation demonstrated a composite pheochromocytoma. Diarrhea stopped immediately after surgery together with a normalization of laboratory parameters. In conclusion, this case report focuses on the rare clinical presentation of secretory diarrhea and electrolyte disturbances in combination with hypokalemic rhabdomyolysis which was caused by a VIP-producing composite pheochromocytoma.

Octreotide reverses shock due to vasoactive intestinal peptide-secreting adrenal pheochromocytoma: A case report and review of literature

“…However, octreotide had little impact on plasma CAT concentration, corroborating previous reports[ 15 , 16 ], which indicate that excessive VIP release may be the most important reason causing shock. On the other hand, the cardiotoxic effects of CATs are chronicled in a number of publications, having linked CAT excess with acute myocarditis and cardiogenic shock[ 17 , 18 ]. This is the most common cause of shock due to pheochromocytoma.…”

Successful Treatment of Hypokalemic Rhabdomyolysis Caused by a Pancreatic VIPoma: A Case Report

“…Early identification, however, can be difficult, depending on the extent of diarrhea. In fact, 16% of patients with pancreatic VIPoma have no WDHA at all [7]. Consequently, diagnosing the tumor requires a high index of suspicion of the condition as well as a serum VIP concentration in excess of 75 pg/ml [8].…”

“…The unregulated overproduction of VIP results in large-volume diarrhea, often severe enough to cause hypokalemia, dehydration, and hypochlorhydria. This watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome caused by VIP-producing tumors is a well-known effect of VIPoma, but progression of the metabolic disturbance to the point of hypokalemic rhabdomyolysis has only been reported on 3 other occasions, with 2 of those cases involving the adrenal gland [6,7]. The only reported case originating in the pancreas also had hepatic metastasis [8].…”