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Watery diarrhoea with a vasoactive intestinal peptide-producing ganglioneuroblastoma
Insights
A rare suprarenal ganglioneuroblastoma caused watery diarrhea and failure to thrive in an infant by producing vasoactive intestinal peptide. Surgical removal of the tumor resolved the symptoms, highlighting the link between neural crest tumors and pediatric secretory diarrhea.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Gastroenterology
Background:
- Vasoactive intestinal peptide (VIP) is a hormone that regulates intestinal secretion.
- Neural crest tumors can rarely secrete VIP, leading to secretory diarrhea.
Observation:
- An 8-month-old boy presented with persistent watery diarrhea, failure to thrive, abdominal distension, hypokalemia, and flushing.
- Elevated serum levels of vasoactive intestinal peptide-like immunoreactivity were detected.
Findings:
- A suprarenal mass was resected, which was histologically diagnosed as a ganglioneuroblastoma.
- Post-operatively, serum VIP levels normalized, and the diarrhea resolved.
- Tumor cells exhibited VIP immunoreactivity and abundant secretory granules.
Implications:
- This case highlights a rare cause of secretory diarrhea in infants due to a neural crest tumor.
- Early diagnosis and surgical resection of such tumors can lead to complete recovery.
- Understanding the clinical features of this syndrome is crucial for timely intervention.
Abstract:
An 8-month-old boy with persistent watery diarrhoea and failure to thrive developed abdominal distension, hypokalaemia, and flushing of the face and trunk. A high concentration of vasoactive intestinal peptide-like immunoreactivity was found in the serum. Soon after resection of a suprarenal mass, the serum level of vasoactive intestinal peptide became normal and the diarrhoea stopped. Histologically the tumour was a ganglioneuroblastoma: the cells showed fluorescence by the indirect immunofluorescence technique with anti-vasoactive intestinal peptide serum. Electron microscopical examination showed abundant secretory granules in the tumour cells. Reports of chronic watery diarrhoea in children due to neural crest tumours are reviewed, with particular respect to the clinical features of the syndrome.