恶性蓝色黑色素瘤

IF 2.3 4区 医学 Q2 DERMATOLOGY
Yo Kaku, Arnaud de la Fouchardière
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引用次数: 0

摘要

恶性蓝色黑素瘤(MBMs)源于蓝色痣和所有相关的皮内黑素细胞增生。它们在G偶联蛋白通路中携带特异的、相互排斥的突变,主要涉及GNAQ或GNA11。其他罕见的遗传驱动因素包括 CYSLTR2 或 PCLB4 突变。最近又增加了 PKC 和 GRM1 基因融合。鳞状上皮细胞瘤好发于头皮部位,表现为在原有病灶内迅速生长的结节。从组织病理学角度看,这些肿瘤位于真皮层和皮下脂肪中,由大结节或扩张的致密片状物组成。常见肿瘤坏死。放大镜下可见大的纺锤形和上皮样黑素细胞,细胞学不典型性高,常有有丝分裂。中央肿块一侧常可见良性蓝色痣或中级蓝色黑素细胞瘤。核 BAP1 免疫活性丧失是一个不良预后因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Malignant blue melanoma.

Malignant blue melanomas arise from blue nevi and all related intradermal melanocytic proliferations. They harbor specific, mutually exclusive mutations in the G-coupled protein pathway, mainly involving GNAQ or GNA11. Other rare genetic drivers include CYSLTR2 or PCLB4 mutations. PKC and GRM1-gene fusions have been recently added to this list. Malignant blue melanomas have a predilection for the scalp area, presenting as rapidly growing nodules within a pre-existing lesion. Histopathologically, these tumors are located in the dermis and subcutaneous fat and consist of large nodules or expanding dense sheets. Tumor necrosis is commonly seen. Large spindle-shaped and epithelioid melanocytes with high-grade cytologic atypia and frequent mitotic figures are at higher magnification. A benign blue nevus or intermediate-grade blue melanocytoma is frequently found on the side of the central mass. Loss of nuclear BAP1 immunoreactivity is a poor prognostic factor.

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来源期刊
Clinics in dermatology
Clinics in dermatology 医学-皮肤病学
CiteScore
4.60
自引率
7.40%
发文量
106
审稿时长
3 days
期刊介绍: Clinics in Dermatology brings you the most practical and comprehensive information on the treatment and care of skin disorders. Each issue features a Guest Editor and is devoted to a single timely topic relating to clinical dermatology. Clinics in Dermatology provides information that is... • Clinically oriented -- from evaluation to treatment, Clinics in Dermatology covers what is most relevant to you in your practice. • Authoritative -- world-renowned experts in the field assure the high-quality and currency of each issue by reporting on their areas of expertise. • Well-illustrated -- each issue is complete with photos, drawings and diagrams to illustrate points and demonstrate techniques.
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