Priyadharsini Nagarajan, Sook Jung Yun, Victor G Prieto
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引用次数: 0
摘要
粘膜黑色素瘤是一种罕见的恶性肿瘤,产生于人体粘膜内表面的上皮细胞。与皮肤黑色素瘤不同,我们目前对粘膜黑色素瘤的了解十分有限。粘膜黑色素瘤的基因改变与皮肤黑色素瘤截然不同,但其致病和促发因素尚不清楚。这些黑色素瘤由于位置隐蔽,通常在晚期才被诊断出来,因此预后较差。目前只有鼻窦和结膜黑色素瘤有专门的粘膜黑色素瘤分期系统。因此,对粘膜黑色素瘤患者,尤其是肛门部位的粘膜黑色素瘤患者进行风险分层具有挑战性。最近的研究表明,对 AJCC 第 8 版皮肤黑色素瘤分期系统稍加修改,就能相当稳妥地对患者进行分组;不过,所提出的 T 分类系统还有待在更大的群体中进行验证。我们总结了粘膜黑色素瘤常见亚型的人口统计学、临床、组织病理学和分子特征,并强调了这一领域的突出需求。
Mucosal Melanoma: Review from a Pathologist Point of View.
Mucosal melanomas are rare malignant tumors arising from the epithelia lining the inner mucosal surfaces of the body. Unlike cutaneous melanoma, we have a limited understanding of mucosal melanomas is currently limited. Mucosal melanomas are characterized by genetic alterations quite distinct from cutaneous melanomas; however, their causative and promoting factors are unknown. These melanomas are characteristically diagnosed at a later stage due to their occult locations, leading to a worse prognosis. Dedicated staging systems for mucosal melanomas exist only for sinonasal and conjunctival melanomas. Therefore, risk stratification of patients with mucosal melanomas, particularly those arising from the anogenital area, is challenging. Recent studies have shown that minor modifications of the AJCC 8th Edition cutaneous melanoma staging system can group patients fairly robustly; however, the proposed T-categorization systems have yet to be validated in larger cohorts. We summarize the demographic, clinical, histopathologic, and molecular features of common subtypes of mucosal melanomas and highlight the outstanding needs in this field.