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Pheochromocytoma producing vasoactive intestinal peptide.
Stephen L Smith1, A L Jackson Slappy, Thomas P Fox
1Section of General Surgery, Mayo Clinic, Jacksonville, Fla 32224, USA. smith.stephen@mayo.edu
Mayo Clinic Proceedings
|January 25, 2002
Summary
A rare case of watery diarrhea syndrome caused by a VIP-producing pheochromocytoma, an adrenal tumor, was observed. Surgical removal of the adrenal mass resolved the patient's symptoms and normalized hormone levels.
Area of Science:
- Endocrinology
- Oncology
- Gastroenterology
Background:
- Watery diarrhea syndrome, characterized by hypokalemia and achlorhydria, is typically linked to neuroendocrine tumors, most commonly of pancreatic origin, secreting vasoactive intestinal peptide (VIP).
- The classical presentation involves secretory diarrhea, electrolyte imbalances, and significant weight loss.
Observation:
- A 78-year-old woman presented with a gradual onset of hypokalemia, watery diarrhea, and weight loss.
- Imaging revealed a left adrenal mass, initially treated medically with a transient response.
- Elevated levels of VIP, pancreatic polypeptide, dopamine, and vanillylmandelic acid were detected.
Findings:
- The adrenal mass was surgically excised and identified as a VIP-producing pheochromocytoma.
- Post-surgical normalization of electrolytes and VIP levels was observed.
- Resolution of watery diarrhea and weight loss occurred after tumor removal.
Implications:
- This case highlights that pheochromocytomas, though rare, can present as a cause of VIP-mediated watery diarrhea syndrome.
- It underscores the importance of considering adrenal origins in the differential diagnosis of neuroendocrine tumors causing secretory diarrhea.
- Successful surgical management of adrenal VIPomas can lead to complete symptom resolution and hormonal normalization.