恶性黑色素瘤/发育不良痣和神经系统肿瘤的家族聚集:肿瘤易感性的原始综合征。

Annales de genetique Pub Date : 1997-01-01
M Bahuau, D Vidaud, M Kujas, A Palangié, B Assouline, M Chaignaud-Lebreton, M Prieur, M Vidaud, J P Harpey, J Lafourcade, B Caille
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引用次数: 0

摘要

这里报告了一个五代家族,其中几个成员在一个病例中发生了恶性黑色素瘤、发育不良痣、各种级别的星形细胞瘤、良性或恶性神经鞘瘤、神经纤维瘤或脑膜瘤。皮肤和神经肿瘤的显著共分离,1型神经纤维瘤病的等位基因的排除,以及已知的遗传性癌症易感性综合征的表型偏离,表明恶性黑色素瘤和中枢/周围神经肿瘤的原始家族易感性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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.

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