Chokoeva A, Tchernev G, Wollina U
Akush Ginekol (Sofiia). 2015;54(2):56-60.
Malignant melanoma of the vulva is a rare disease with aggressive behavior and poor prognosis. It consist < 5% of all cases of melanoma in females, as the ratio of its manifestation, compared with the cutaneous melanoma is 1:71. Higher risk of developing melanoma of the vulva is established in white women, as the peak of the incidence is between 60 and 70 years of age. Clinically, MM of the vulva manifests as asymptomatic pigmented, rarely a pigmented lesion, as the usual clinical form is superficial spreading MM and much less common nodular MM, which is associated with a poorer prognosis in. general. The diagnosis is confirmed by histological examination. Conduction of PCR and DNA analysis for detection of BRAF mutations, NRAS mutations and KIT amplification is also appropriate. Advanced age, black race, tumor size, tumor thickness, ulceration, presence of satellite lesions, involvement of adjacent organs (vagina, urethra), and the presence of regional or distant metastases are identified as the most important prognostic markers. Radical wide excision followed by bilateral lymphadenectomy id considered as the optimal therapeutic approach.
外阴恶性黑色素瘤是一种罕见疾病，具有侵袭性且预后较差。它占女性所有黑色素瘤病例的不到5%，其发病率与皮肤黑色素瘤相比为1:71。白人女性患外阴黑色素瘤的风险更高，发病高峰在60至70岁之间。临床上，外阴恶性黑色素瘤表现为无症状色素沉着，很少为色素性病变，常见临床类型为浅表扩散型恶性黑色素瘤，结节型恶性黑色素瘤则少见得多，总体而言其预后较差。诊断需经组织学检查确认。进行PCR和DNA分析以检测BRAF突变、NRAS突变和KIT扩增也是合适的。高龄、黑人种族、肿瘤大小、肿瘤厚度、溃疡、卫星灶的存在、邻近器官（阴道、尿道）受累以及区域或远处转移的存在被确定为最重要的预后标志物。根治性广泛切除加双侧淋巴结清扫被认为是最佳治疗方法。
