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B-K痣综合征。皮肤和眼部恶性黑色素瘤。

B-K mole syndrome. Cutaneous and ocular malignant melanoma.

作者信息

Abramson D H, Rodriguez-Sains R S, Rubman R

出版信息

Arch Ophthalmol. 1980 Aug;98(8):1397-9. doi: 10.1001/archopht.1980.01020040249006.

Abstract

A 51-year-old white man had the B-K mole syndrome (multiple large atypical cutaneous nevi of the upper part of the trunk and extremities, inherited as an autosomal dominant trait, and thought to be more susceptible to malignant transformation), so named after two patients in whom the syndrome was first observed. Two cutaneous malignant melanomas (thigh and back) and an ocular malignant melanoma (ciliary body and iris) simultaneously developed. Patients with B-K mole syndrome have been known to have a very high risk for the development of cutaneous melanoma (including multiple primary cutaneous melanomas) and multiple primary malignancies. There may be a propensity in these patients for development of ocular melanomas.

摘要

一名51岁的白人男性患有B-K痣综合征(躯干上部和四肢有多个大型非典型皮肤痣,呈常染色体显性遗传,被认为更易发生恶性转化),该综合征因首次观察到的两名患者而得名。同时出现了两处皮肤恶性黑色素瘤(大腿和背部)和一处眼部恶性黑色素瘤(睫状体和虹膜)。已知患有B-K痣综合征的患者发生皮肤黑色素瘤(包括多发性原发性皮肤黑色素瘤)和多发性原发性恶性肿瘤的风险非常高。这些患者可能有发生眼部黑色素瘤的倾向。

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