Coexistent neuroblastoma and Hirschsprung's disease —Another manifestation of the neurocristopathy?

摘要

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 developmental pathology, i. e., Hirschsprung's representing a deficiency and neuroblastoma representing malignant proliferation of cells of neural crest ori