原发性黏膜黑素瘤是一种起源于呼吸道,消化道和泌尿生殖系统黑素细胞的潜在危及生命的高度恶性肿瘤,具有明显的生物学和临床特征。与皮肤恶性黑素瘤相比,其发病机制、危险因素、分子生物学特征性等因素尚待确定。虽然几乎任何黏膜表面都可以发生黑素瘤,但是大多数起源于头颈部、肛门直肠、或者是外阴阴道的黏膜。起病隐匿,早期缺乏典型的特征,临床表现多样,易于漏诊及误诊,预后不良。手术仍然是黏膜黑素瘤的主要治疗手段。
As a potentially life-threatening malignancy arising from melanocytes located in mucosal membranes lining respiratory, gastrointestinal and urogenital tract, primary mucosal melanomas have distinct biological and clinical features. Unlike cutaneous malignant melanoma, the pathogenesis, risk factors and molecular biology characteristics of primary mucosal melanomas remain unanswered. Although they can arise in almost any part of mucosal membranes, a majority of them originate from the mucosa of the head and neck, anorectum or vulva and vagina. Most of mueosal melanomas occur in occult sites, which together with the lack of early and specific signs contributes to the misdiagnosis, missed diagnosis and poor prognosis of mucosal melanomas. Surgery is still the mainstay of treatment for mucosal melanomas.