Elsevier

Human Pathology

Volume 5, Issue 4, July 1974, Pages 409-429
Human Pathology

The neurocristopathies: A unifying concept of disease arising in neural crest maldevelopment

https://doi.org/10.1016/S0046-8177(74)80021-3Get rights and content

Abstract

Pheochromocytoma, neuroblastoma, neurofibromatosis, medullary carcinoma of the thyroid, carcinoid tumors, Hirschsprung's disease, and nonchromaffin paragangliomas are discussed and analysed in terms of their proposed neural crest origins. Combinations and permutations of these entities form discrete syndromes, all of which tend to be embryogenetically related as the neurocristopathies. Other peculiar interrelationships are shown, emphasizing their common neuroectodermal origin. The wide distribution and multipotentialities of the migratory derivatives of the neural crest and their susceptibilities to teratogenic, oncogenic, and mutagenic influences are discussed.

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    *

    Professor of Pathology, McGill University. Director of Pathology, The Montreal Children's Hospital, Montreal, Quebec.

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