Department of Obstetrics and Gynecology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA 90095, USA.
Department of Hematology and Oncology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA 90095, USA.
Curr Oncol. 2021 Oct 21;28(6):4264-4272. doi: 10.3390/curroncol28060362.
Primary mucosal melanomas of the female genital tract account for one percent or less of all cases of melanoma with even fewer originating in the clitoris. Given the rarity of diagnosis of clitoral melanoma, there is a paucity of data guiding management. There is no supporting evidence that radical vulvectomy (with or without inguinal lymphadenopathy) is associated with improved disease-free or overall survival compared to partial vulvectomy or wide local excision. Additionally, there is no data to evaluate the role of sentinel lymph node biopsy or extensive lymphadenectomy in clitoral melanoma, however previous evidence demonstrates the utility of regional lymph node sampling in predicting survival in women with female genital tract mucosal melanoma. Adjuvant therapy considerations are often extrapolated from their use in treating cutaneous melanomas, including immune checkpoint inhibitors and other immunotherapy agents. Adjuvant radiation therapy has limited utility except in cases of bulky, unresectable disease, or when inguinal lymph nodes are positive for metastasis. The 52 year-old patient presented in this review was diagnosed with locally invasive advanced stage clitoral melanoma presenting as an exophytic clitoral mass. She underwent diagnostic primary tumor resection, which demonstrated ulcerative melanoma with spindle cell features extending to a Breslow depth of at least 28 mm. She subsequently underwent secondary wide local excision with groin sentinel lymph node biopsy, and adjuvant treatment with pembrolizumab. This article also emphasizes the importance of a multidisciplinary team involving gynecologic oncology, medical oncology, radiology, and pathology for management of this rare type of primary mucosal melanoma of the female genital tract.
女性生殖道原发性黏膜黑色素瘤占所有黑色素瘤病例的 1%或更少，而起源于阴蒂的黑色素瘤则更少。由于诊断阴蒂黑色素瘤的罕见性，指导管理的资料很少。没有证据表明根治性外阴切除术（伴或不伴腹股沟淋巴结病）与部分外阴切除术或广泛局部切除术相比能改善无病生存率或总生存率。此外，尚无数据评估前哨淋巴结活检或广泛淋巴结切除术在阴蒂黑色素瘤中的作用，但以前的证据表明区域淋巴结取样可预测女性生殖道黏膜黑色素瘤患者的生存率。辅助治疗的考虑因素通常是从其在治疗皮肤黑色素瘤中的应用中推断出来的，包括免疫检查点抑制剂和其他免疫治疗药物。辅助放疗的作用有限，除非是巨大的、不可切除的疾病，或腹股沟淋巴结有转移。本综述中介绍的 52 岁患者被诊断为局部侵袭性晚期阴蒂黑色素瘤，表现为外生性阴蒂肿块。她接受了诊断性原发性肿瘤切除术，显示溃疡性黑色素瘤，具有梭形细胞特征，Breslow 深度至少为 28mm。随后她接受了二次广泛局部切除和腹股沟前哨淋巴结活检，并接受了 pembrolizumab 辅助治疗。本文还强调了多学科团队的重要性，该团队涉及妇科肿瘤学、医学肿瘤学、放射学和病理学，以管理这种罕见的女性生殖道原发性黏膜黑色素瘤。
