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Vasoactive intestinal polypeptide-secreting ganglioneuromatosis affecting the entire colon and rectum
F J Rescorla1, D W Vane, J F Fitzgerald
1Department of Surgery, Indiana University School of Medicine, Indianapolis.
Insights
A rare case of watery diarrhea in a child was linked to a widespread VIP-secreting tumor. Surgical removal of the tumor led to symptom resolution and normalized VIP levels.
Area of Science:
- Pediatric Gastroenterology
- Surgical Oncology
- Endocrinology
Background:
- Vasoactive intestinal polypeptide (VIP)-secreting tumors are rare, typically presenting in children with secretory diarrhea.
- Ganglioneuromatosis is a neoplastic proliferation of ganglion cells and their supporting elements.
Observation:
- A 7-year-old boy presented with severe watery diarrhea, hypokalemia, and hypochlorydia.
- Diagnostic evaluation revealed ganglioneuromatosis involving the entire colon and rectum, secreting VIP.
Findings:
- The patient's symptoms resolved completely after a proctocolectomy.
- Post-operative serum VIP levels normalized, confirming the tumor as the source of excess VIP.
Implications:
- This case represents the first reported instance of VIP-secreting ganglioneuromatosis affecting the entire colon and rectum in a child.
- Highlights the importance of considering extensive gastrointestinal involvement in VIP-secreting tumors, even in pediatric cases.
- Surgical resection can be curative for such extensive tumors.
Abstract:
This report describes a 7-year-old boy presenting with watery diarrhea, hypokalemia, and hypochlohydria associated with vasoactive intestinal polypeptide (VIP)-secreting ganglioneuromatosis involving the entire colon and rectum. The child's symptoms resolved following proctocolectomy, and the VIP levels returned to normal. Although 55 previous children have been reported with VIP-secreting tumors, this case is the first involving the entire colon and rectum.