Falcicchio Giovanni, Vinci Lorenzo, Cicinelli Ettore, Loizzi Vera, Arezzo Francesca, Silvestris Erica, Resta Leonardo, Serio Gabriella, Cazzato Gerardo, Mastronardi Marialuigia, Mongelli Michele, Marinaccio Marco, Cormio Gennaro
Section of Gynecology and Obstetrics, Department of Biomedical Sciences and Human Oncology (DIMO), University of Bari "Aldo Moro", 70124 Bari, Italy.
Section of Gynecology and Obstetrics, Interdisciplinar Department of Medicine, University of Bari "Aldo Moro", 70124 Bari, Italy.
Cancers (Basel). 2022 Oct 25;14(21):5217. doi: 10.3390/cancers14215217.
Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar neoplasms. VM primarily affects older Caucasian women and its relationship to sun exposure is undefined. Diagnosis is defined by biopsy but many clinical, dermatoscopic, and confocal microscopic features can guide doctors. The molecular profile is characterized by the KIT mutation, revealed by all of the technologies that are used (classical sequencing, next-generation sequencing, and immunohistochemical staining). BRAF and NRAS mutations are also common in VM. All of these mutations are possible therapeutic targets. Today, surgery remains the first treatment choice for primary VM. The role of neoadjuvant and adjuvant therapy is scarce and the treatment of relapses is widely debated.
恶性黑色素瘤是一种影响所有皮肤部位的致命疾病。其中，外阴黑色素瘤（VM）是一种罕见的妇科疾病，占所有外阴肿瘤的5%。VM主要影响老年白人女性，其与阳光照射的关系尚不清楚。诊断通过活检确定，但许多临床、皮肤镜和共聚焦显微镜特征可指导医生。分子特征以KIT突变为主，所有使用的技术（经典测序、下一代测序和免疫组织化学染色）均揭示了该突变。BRAF和NRAS突变在VM中也很常见。所有这些突变都是可能的治疗靶点。如今，手术仍然是原发性VM的首选治疗方法。新辅助和辅助治疗的作用尚不明确，复发的治疗也存在广泛争议。
