Hepatic cholera: a case report with literature review

Abstract

Abstract Background Vasoactive intestinal peptide-secreting tumors (VIPomas), also known as WDHA syndrome (or “pancreatic cholera”), are rare neuroendocrine tumors (NETs) with an incidence of 0.05 to 2%. It secretes excessive amounts of VIP, leading to watery diarrhea, hypokalemia, and hypochlorhydria. In adults, they occur most commonly between the ages of 30 and 50 years and are mostly intrapancreatic, occurring in the tail of the pancreas, and rarely located in other segments of the gastrointestinal tract, such as the adrenal glands (35%), paraspinal retroperitoneal ganglia (30–35%), posterior mediastinum (20%), head and neck (1–5%), pelvis (2–3%), and rarely (1%) thymus, lung, kidney, anterior mediastinum, and liver. Case presentation Our patient, a 22-year-old man, was admitted with recurrent episodes of large-volume chronic watery diarrhea, generalized muscle weakness, and significant weight loss. After a complete workup, a solitary primary hepatic VIPoma was diagnosed. The patient underwent surgical resection of the liver lesion. Following surgery, there was immediate regression of diarrhea and electrolyte abnormalities, with no recurrence at the 1-year follow-up. Conclusion Neuroendocrine tumors, particularly VIPomas, are rare and mostly intrapancreatic. Solitary primary hepatic VIPoma is extremely rare, and to the best of our knowledge, only four cases without any evidence of a pancreatic lesion have been reported. Diagnosis remains challenging, as it is often not detected on conventional imaging. The prognosis mostly depends on tumor grade, stage, and surgical resectability.

Keywords

• Neuroendocrine tumors
• Vasoactive intestinal peptide-secreting tumors
• Primary hepatic VIPoma
• VIPomas
• WDHA syndrome
• Pancreatic cholera