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Case Reports
. 2014 Jul 31:14:553.
doi: 10.1186/1471-2407-14-553.

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

Jingjing Jiang, Li Zhang, Zhaodi Wu, Zhilong Ai, Yingyong Hou  1 , Zhiqiang Lu, Xin Gao
Affiliations
Case Reports

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

Jingjing Jiang et al. BMC Cancer. .

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.

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Figures

Figure 1
Figure 1
A PET-CT scan demonstrating a large, well defined adrenal mass. A large hyper-metabolic mass was located at the right adrenal region. The hypodense areas at the center indicated necrosis. Massive fluid collection could be found in the colon.
Figure 2
Figure 2
Pathology slides of the resected specimen with different stains. A: Hematoxylin and eosin stained section of the adrenal mass demonstrating adipose tissues infiltrated by tumor cells (×100). B: Immunohistochemical detection of synaptophysin in the tumor cells (×100). C: Immunohistochemical detection of chromogranin A in the tumor cells (×100). D: Immunohistochemical detection of VIP-positive cells (×200).
See this image and copyright information in PMC

References

    1. Verner JV, Morrison AB. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med. 1958;25(3):374–380. doi: 10.1016/0002-9343(58)90075-5. - DOI - PubMed
    1. Loehry CA, Kingham JG, Whorwell PJ. Watery diarrhoea and hypokalaemia associated with a phaeochromocytoma. Postgrad Med J. 1975;51(596):416–419. doi: 10.1136/pgmj.51.596.416. - DOI - PMC - PubMed
    1. Cooperman AM, Desantis D, Winkelman E, Farmer R, Eversman J, Said S. Watery diarrhea syndrome. Two unusual cases and further evidence that VIP is a humoral mediator. Ann Surg. 1978;187(3):325–328. doi: 10.1097/00000658-197803000-00020. - DOI - PMC - PubMed
    1. Fisher BM, MacPhee GJ, Davies DL, McPherson SG, Brown IL, Goldberg A. A case of watery diarrhoea syndrome due to an adrenal phaeochromocytoma secreting vasoactive intestinal polypeptide with coincidental autoimmune thyroid disease. Acta Endocrinol (Copenh) 1987;114(3):340–344. - PubMed
    1. Matta MK, Prorok JJ, Trimpi HD, Sheets JA, Stasik JJ, Jr, Khubchandani IT. WDHA syndrome caused by pheochromocytoma: report of a case. Dis Colon Rectum. 1978;21(4):297–301. doi: 10.1007/BF02586708. - DOI - PubMed
Pre-publication history
    1. The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/14/553/prepub

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