Watery diarrhea, hypokalemia and achlorhydria syndrome due to an adrenal pheochromocytoma

Abstract

Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma, which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.

Figure 1

A: Enhanced abdominal CT scan showing a right adrenal tumor (arrows); B: Cut section of the tumor showing variegated white and red-brown areas; C: Immunohistochemical detection of chromogranin A in the tumor cells (× 200); D: Immunohistochemical detection of VIP-positive cells. Stained cells were scattered throughout the tumor tissue (× 300).