Abstract
BackgroundA 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 presentationA 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.ConclusionWe report an extremely rare case of pheochromocytoma causing WDHA syndrome and uremia, which the patient completely recovered from after tumor resection.
Highlights
A rare syndrome of watery diarrhea, hypokalemia and achlorhydria (WDHA) is usually caused by pancreatic endocrine tumors that secrete excessive vasoactive intestinal polypeptide (VIP)
A rare syndrome of watery diarrhea associated with hypokalemia and achlorhydria (WDHA) due to hypersecretion of VIP was described initially by Verner and Morrison in 1958 [1]
We describe a case of WDHA caused by a VIP-positive pheochromocytoma
Summary
Vasoactive intestinal peptide (VIP) is a 28-amnio acid peptide that may cause secretory diarrhea when overproduced by activating adenylate cyclase. Case presentation A 45-year old man presented with persistent and progressive watery diarrhea for half a year He was initially admitted to a local hospital because he suddenly lost consciousness, during which his blood pressure was unmeasurable. Arterial blood gas tests indicated metabolic acidosis and hypoxia (pH 7.16, HCO3 10 mmol/L, PO2 70%) He was intubated, maintained on hemodialysis and treated with fluid and antibiotics intravenously. The patient underwent a combined surgery, which removed the adrenal mass and the left lobe of the thyroid gland simultaneously. His daily urine increased gradually and serum Cr lowered into a normal range without dialysis. Plasma NMN, serum PTH, calcitonin and electrolytes all were back to normal (Table 1). The patient has been followed for one year, showing no sign of recurrence
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
