女性生殖道黑色素瘤(FGTM)是一种罕见的侵袭性黑色素细胞恶性肿瘤,其不同解剖部位的临床病理和预后特征尚未得到充分描述。我们回顾性分析并比较了2005年1月至2020年12月间纳入的原发性下生殖道黑色素瘤患者的临床病理资料和生存结果。我们确定了95例FGTM患者,其中46人患有外阴黑素瘤(VuM),43人患有阴道黑素瘤(VaM),6例宫颈黑色素瘤(CM)。所有95例患者的临床特点,包括症状,单个或多个原发性病变,临床分期,手术,62例原发性未治疗病例的组织病理学特征,包括色素沉着,主要细胞学,组织学模式,有丝分裂图,和VuM的肿瘤浸润淋巴细胞,VaM,和CM,差异显著。相比之下,只有分子变化的趋势差异是明显的(p=0.077)。5年的疾病特异性生存率(DSS)为30.7%(46.5%,25.6%,VuM为44.4%,VaM和CM,分别)。71例(85.5%)患者出现FGTM复发。首次复发的中位时间为11个月,VaM比VM和CM更早复发(VuM为16、6和10个月,VaM,和CM,分别,p=0.038)。对50例患者的单因素分析揭示了疾病特异性生存(DSS)的负面因素,包括阴道的位置和溃疡的存在,和无复发生存(RFS)的负面因素,包括多发性病变,溃疡的存在,和淋巴管浸润的存在。多个病变与DSS呈边缘性相关。对50例患者的多变量Cox回归分析显示,溃疡的存在与较短的DSS和RFS有关(是与不,危险比分别=2.400和2.716)。阴道位置与DSS显著相关(危险比=2.750,p=0.024)。总之,外阴,阴道,宫颈黑色素瘤的临床病理特征以及与DSS和RFS的关系可能有所不同。溃疡和阴道位置与较短的DSS显著相关,溃疡与FGTM复发风险增加相关。
Female genital tract melanoma (FGTM) is a rare and aggressive melanocytic malignancy, and its clinico-pathological and prognostic features at different anatomic sites have not yet been fully described. We retrospectively analyzed and compared the clinico-pathological data and survival outcomes of patients with primary lower genital tract melanoma enrolled between January 2005 and December 2020. We identified 95 patients with FGTM, of whom 46 had vulvar melanomas (VuM), 43 had vaginal melanomas (VaM), and six had cervical melanomas (CM). The clinical characteristics of all 95 cases, including symptoms, single or multiple primary lesions, clinical stage, surgery, and histopathological characteristics of 62 primary untreated cases, including pigmentation, predominant cytology, histological pattern, mitotic figures, and tumor-infiltrating lymphocytes of VuM, VaM, and CM, differed significantly. In comparison, only trend differences in molecular alternations were evident (p = 0.077). Disease-specific survival (DSS) was 30.7% at 5 years (46.5%, 25.6%, and 44.4% for VuM, VaM and CM, respectively). Seventy-one (85.5%) patients experienced FGTM recurrence. The median time to the first recurrence was 11 months, and VaM recurred earlier than VM and CM (16, 6, and 10 months for VuM, VaM, and CM, respectively, p = 0.038). A univariate analysis of 50 cases revealed the negative factors of disease-specific survival (DSS), including the location of the vagina and the presence of ulceration, and the negative factors of recurrence-free survival (RFS), including multiple lesions, the presence of ulceration, and the presence of lymphovascular invasion. Multiple lesions showed a borderline correlation with DSS. A multivariate Cox regression analyses of 50 cases revealed that the presence of ulceration was associated with shorter DSS and RFS (yes vs. no, Hazard Ratio = 2.400 and 2.716, respectively). Vaginal location showed a significant correlation with DSS (Hazard Ratio = 2.750, p = 0.024). In conclusion, vulval, vaginal, and cervical melanomas may differ in terms of their clinico-pathological features and associations with DSS and RFS. Ulceration and vaginal location were significantly associated with shorter DSS, and ulceration was associated with an increased risk of FGTM recurrence.