Coexistent neuroblastoma and Hirschsprung's disease--another manifestation of the neurocristopathy?
Abnormalities of neural crest development may be manifested in a wide variety of ways ranging from von Recklinghausen's disease to Sipple's syndrome -- pheochromocytoma and medullary thyroid carcinoma. We report here yet another manifestation -- coexistent multiple neuroblastomas and Hirschsprung's disease in a newborn. In a review of the English literature we have found the combination of neuroblastoma and Hirschsprung's disease recorded only once previously [2]. However, the relationship between the two diseases was not discussed. The two diseases may be at the extremes of a spectrum of neural crest development pathology, i.e., Hirschsprung's representing a deficiency and neuroblastoma representing malignant proliferation of cells of neural crest origin.
D000008:Abdominal Neoplasms / Q000150:complications; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008531:Megacolon / Q000150:complications*; D009421:Nervous System Malformations; D009432:Neural Crest; D009447:Neuroblastoma / Q000150:complications*