title: 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.
authors.affiliation: Laboratoire de Génétique Moléculaire, Faculté des Sciences Pharmaceutiques et Biologiques de Paris, Université Paris V, France.
authors.forename: M
authors.initials: M
authors.lastname: Bahuau
authors.forename: D
authors.initials: D
authors.lastname: Vidaud
authors.forename: M
authors.initials: M
authors.lastname: Kujas
authors.forename: A
authors.initials: A
authors.lastname: Palangié
authors.forename: B
authors.initials: B
authors.lastname: Assouline
authors.forename: M
authors.initials: M
authors.lastname: Chaignaud-Lebreton
authors.forename: M
authors.initials: M
authors.lastname: Prieur
authors.forename: M
authors.initials: M
authors.lastname: Vidaud
authors.forename: J P
authors.initials: JP
authors.lastname: Harpey
authors.forename: J
authors.initials: J
authors.lastname: Lafourcade
authors.forename: B
authors.initials: B
authors.lastname: Caille
mesh_terms: D000328:Adult; D002648:Child; D004416:Dysplastic Nevus Syndrome / Q000235:genetics*; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007621:Karyotyping; D008297:Male; D008545:Melanoma / Q000235:genetics*; D009423:Nervous System Neoplasms / Q000235:genetics*; D010375:Pedigree; D011110:Polymorphism, Genetic; D012878:Skin Neoplasms / Q000235:genetics*; D013577:Syndrome