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Familial aggregation of malignant melanoma/dysplastic naevi and tumours of the nervous system: an original syndrome of tumour proneness.

Abstract
A five-generation family is here reported in which several members developed malignant melanoma, dysplastic naevi, astrocytoma in all grades, benign or malignant schwannoma, neurofibroma, or meningioma in a single instance. Significant cosegregation of skin and nervous tumours, preclusion of allelism to type 1 neurofibromatosis and phenotypic departure from known syndromes of hereditary proneness to cancer make one suggest an original familial predisposition to both malignant melanoma and central/peripheral nervous tumours.
AuthorsM Bahuau, D Vidaud, M Kujas, A Palangié, B Assouline, M Chaignaud-Lebreton, M Prieur, M Vidaud, J P Harpey, J Lafourcade, B Caille
JournalAnnales de genetique (Ann Genet) Vol. 40 Issue 2 Pg. 78-91 ( 1997) ISSN: 0003-3995 [Print] Netherlands
PMID9259954 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Topics
  • Adult
  • Child
  • Dysplastic Nevus Syndrome (genetics)
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Karyotyping
  • Male
  • Melanoma (genetics)
  • Nervous System Neoplasms (genetics)
  • Pedigree
  • Polymorphism, Genetic
  • Skin Neoplasms (genetics)
  • Syndrome

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