Landthaler M, Braun-Falco O
Med Klin. 1979 Mar 9;74(10):353-7.
The existence of familial-hereditary malignant melanoma can be accepted for sure. Its frequency is estimated with 1 to 7% of all malignant melanomas. An autosomal-dominant mode of inheritance with reduced penetrance or a polygenetic mode of inheritance are discussed. Patients with numerous inherited, clinical and histological atypical naevi, the so called BK-moles, are reported to have a high incidence of malignant melanoma. Hereditary malignant melanomas begin early in life, have a high frequency of multiple primary lesions and show the coincidence of other malignant tumors in this families. Men are affected as often as women. Five own families are described and a review of the literature is given.
家族遗传性恶性黑色素瘤的存在是可以确定的。其发生率估计占所有恶性黑色素瘤的1%至7%。目前正在讨论具有降低外显率的常染色体显性遗传模式或多基因遗传模式。据报道，患有大量遗传性、临床和组织学非典型痣（即所谓的BK痣）的患者患恶性黑色素瘤的几率很高。遗传性恶性黑色素瘤发病较早，多原发性损害的发生率高，且在这些家族中还可见其他恶性肿瘤同时存在。男性和女性受影响的几率相同。本文描述了五个自家系并对文献进行了综述。
