Coexistent neuroblastoma and Hirschsprung's disease--another manifestation of the neurocristopathy?

Pediatr Radiol. 1979 Jul 24;8(3):161-3. doi: 10.1007/BF00973826.


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.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Neoplasms / complications
  • Humans
  • Infant, Newborn
  • Male
  • Megacolon / complications*
  • Nervous System Malformations
  • Neural Crest
  • Neuroblastoma / complications*