Familial aggregation of malignant melanoma/dysplastic naevi and tumours of the nervous system: an original syndrome of tumour proneness.
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.
Bahuau
M
M
Laboratoire de Génétique Moléculaire, Faculté des Sciences Pharmaceutiques et Biologiques de Paris, Université Paris V, France.
Vidaud
D
D
Kujas
M
M
Palangié
A
A
Assouline
B
B
Chaignaud-Lebreton
M
M
Prieur
M
M
Vidaud
M
M
Harpey
J P
JP
Lafourcade
J
J
Caille
B
B
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