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Vasoactive intestinal peptide secreting tumors of childhood
Insights
A rare ganglioneuroma in a child caused severe symptoms like diarrhea and hypertension due to elevated vasoactive intestinal peptide (VIP) and norepinephrine (NE). Surgical removal cured the child, confirming VIP
Area of Science:
- Pediatric Endocrinology
- Surgical Oncology
- Neuroendocrinology
Background:
- Ganglioneuromas, rare neurocristal tumors, can secrete vasoactive substances.
- Pediatric failure to thrive can be associated with complex gastrointestinal and endocrine disturbances.
Observation:
- A 2-year-old boy presented with failure to thrive, watery diarrhea, abdominal distention, hypokalemia, metabolic acidosis, hypertension, and sweating.
- A calcified right lower quadrant mass was identified, with elevated blood levels of vasoactive intestinal peptide (VIP) and norepinephrine (NE).
Findings:
- Presurgical management with phenoxybenzamine hydrochloride and metyrosine prevented expected postoperative hypotension.
- Resection of the benign ganglioneuroma led to immediate symptom resolution and normalization of VIP and NE levels.
Implications:
- This case highlights the potential for benign ganglioneuromas to cause significant systemic effects through hormone secretion.
- The findings reinforce the role of VIP in mediating the diarrhea associated with certain neurocristal tumors.
- Effective presurgical and surgical management can lead to complete recovery in children with functioning ganglioneuromas.
Abstract:
A 2-year-old boy with failure to thrive, watery diarrhea, abdominal distention, hypokalemia, metabolic acidosis, and episodes of hypertension and sweating was found to have a calcified right lower quadrant mass. Blood levels of vasoactive intestinal peptide (VIP) and norepinephrine (NE) were elevated. Presurgical management with phenoxybenzamine hydrochloride and metyrosine was associated with an absence of expected postoperative hypotension, and resection of a benign ganglioneuroma resulted in prompt relief of all symptoms and return to normal of VIP and NE levels. Evidence supports the theory that VIP is the substance responsible for the diarrhea that accompanies some neural crest tumors.