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Mediastinal ganglioneuroblastoma-secreting vasoactive intestinal peptide causing secretory diarrhoea

Khaled Husain1, Elizabeth Thomas, Zeinab Demerdash

  • 1Department of Paediatric Gastroentrology and Hepatology, Ministry of Health, Kuwait.

Arab Journal of Gastroenterology : the Official Publication of the Pan-Arab Association of Gastroenterology
|June 21, 2011
PubMed

Insights

A rare ganglioneuroblastoma secreting vasoactive intestinal peptide (VIP) caused severe secretory diarrhea in an infant. Surgical resection of the tumor led to complete recovery, indicating a favorable prognosis for resectable cases.

Area of Science:

  • Pediatric Oncology
  • Endocrinology
  • Gastroenterology

Background:

  • Mediastinal tumors are rare in children.
  • Ganglioneuroblastomas can secrete vasoactive intestinal peptide (VIP).
  • VIP-induced secretory diarrhea is a known but uncommon paraneoplastic syndrome.

Observation:

  • An 18-month-old girl presented with prolonged diarrhea, abdominal distension, and weight loss.
  • Investigations revealed secretory diarrhea with electrolyte imbalances (hypokalemia, hyponatremia, hypochloremia) and metabolic acidosis.
  • Elevated VIP levels were detected, with normal glucagon and gastrin levels.

Findings:

  • A right upper zone mediastinal mass was identified on chest X-ray and CT scan.
  • The mass was confirmed to be a ganglioneuroblastoma.
  • Surgical resection of the tumor resulted in the resolution of symptoms.

Implications:

  • This case highlights ganglioneuroblastoma as a treatable cause of VIP-secreting secretory diarrhea in children.
  • Early diagnosis and surgical intervention are crucial for favorable outcomes.
  • VIP-secreting tumors, even when malignant, can have a good prognosis if completely resectable.

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