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Open Access Highly Accessed Case report

A rare case of watery diarrhea, hypokalemia and achlorhydria syndrome caused by pheochromocytoma

Jingjing Jiang1, Li Zhang2, Zhaodi Wu3, Zhilong Ai4, Yingyong Hou5*, Zhiqiang Lu1* and Xin Gao1

Author Affiliations

1 Department of Endocrinology and Metabolism, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, P.R. China

2 Department of Urology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, P.R. China

3 Department of Gastroenterology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, P.R. China

4 Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, P.R. China

5 Department of Pathology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, P.R. China

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BMC Cancer 2014, 14:553  doi:10.1186/1471-2407-14-553

Published: 31 July 2014

Abstract

Background

A rare syndrome of watery diarrhea, hypokalemia and achlorhydria (WDHA) is usually caused by pancreatic endocrine tumors that secrete excessive vasoactive intestinal polypeptide (VIP). Here we report a rare case of WDHA caused by a pheochromocytoma.

Case presentation

A 45-year old male presented with persistent and progressive watery diarrhea for half a year, and was treated with dialysis due to azotemia, hypokalemia, hypercalcemia and metabolic acidosis. A right adrenal mass was found by ultrasonography, and Positron Emission Tomography-Computed Tomography (PET-CT) showed the tumor was hyper-metabolic. Levels of plasma normetanephrine (NMN) and serum chromogranin A (CgA) were significantly elevated. Immunohistochemistry analysis of the adrenal tumor was strongly positive for CgA, synaptophysin and VIP. The patient fully recovered from WDHA syndrome soon after surgery, as reflected in that diarrhea stopped, levels of plasma NMN, serum CgA, and electrolytes returned to normal thus no dialysis was needed. The patient remained disease free in a 12-months follow-up period.

Conclusion

We report an extremely rare case of pheochromocytoma causing WDHA syndrome and uremia, which the patient completely recovered from after tumor resection.

Keywords:
Vasoactive intestinal polypeptide; Pheochromocytoma; Hypercalcemia; Bone metabolism