外阴原发性恶性黑色素瘤——一种用于模拟非紫外线相关黑色素瘤发生的侵袭性肿瘤。
Primary malignant melanoma of the vulva--an aggressive tumor for modeling the genesis of non-UV light-associated melanomas.
作者信息
Ragnarsson-Olding Boel K
机构信息
Department of Oncology, Radiumhemmet, Karolinska University Hospital, Stockholm, Sweden.
出版信息
Acta Oncol. 2004;43(5):421-35. doi: 10.1080/02841860410031372.
Malignant melanomas appear in such sun-shielded areas as the vulva, challenging conventional knowledge that they are associated with UV radiation. Based on 1442 patients with vulvar melanomas the tumors' epidemiology, clinical manifestations, histopathology, molecular genetics, treatment strategies, and prognosis were surveyed. Despite their sun-shielded location and rare incidence, vulvar melanomas were, on average, more dense than melanomas on the body surface and nearly the density of melanomas in chronically sun-exposed skin of the head and neck. Vulvar melanomas differed markedly from cutaneous melanomas, as evidenced by histopathological lesions and molecular genetics. Most melanomas were located on the glabrous skin as opposed to the hairy skin within the vulva and differed significantly in biological properties. The prognosis for the patients was poor, and in the 11 largest studies of surgical strategies, none offered a significant survival advantage. Tumor thickness and ulceration were usually significant predictors of (poor) prognosis in multivariate analyses along with macroscopic amelanosis, angioinvasion or DNA non-diploidy in some reports. Clear-cut biological differences between vulvar and cutaneous melanomas and between melanomas within different vulvar sites provide new paths for extensive research on melanomagenesis and for potential therapies. Additionally, studies of vulvar and other extracutaneous melanomas should characterize subgroups of cutaneous melanomas and identify their cause(s), which are apparently not linked to UV radiation.
恶性黑色素瘤出现在诸如外阴等防晒部位,这对它们与紫外线辐射相关的传统认知提出了挑战。基于1442例外阴黑色素瘤患者,对肿瘤的流行病学、临床表现、组织病理学、分子遗传学、治疗策略及预后进行了调查。尽管外阴黑色素瘤位于防晒部位且发病率较低,但平均而言,其密度高于体表黑色素瘤,接近头颈部长期暴露于阳光下皮肤的黑色素瘤密度。外阴黑色素瘤在组织病理学病变和分子遗传学方面与皮肤黑色素瘤明显不同。大多数黑色素瘤位于外阴无毛皮肤而非有毛皮肤,且生物学特性差异显著。患者预后较差,在11项关于手术策略的最大规模研究中,无一显示出显著的生存优势。在多变量分析中,肿瘤厚度和溃疡通常是(不良)预后的重要预测因素,在一些报告中,宏观无色素沉着、血管侵犯或DNA非二倍体也是如此。外阴黑色素瘤与皮肤黑色素瘤之间以及不同外阴部位的黑色素瘤之间明显的生物学差异为黑色素瘤发生机制的广泛研究和潜在治疗提供了新途径。此外,对外阴及其他皮肤外黑色素瘤的研究应明确皮肤黑色素瘤的亚组并确定其病因,这些病因显然与紫外线辐射无关。