Pomerantz Tali, Rubin Nicole J, Karnezis Anthony N, Zhao Xiao, Brooks Rebecca
Department of Obstetrics and Gynecology, Division of Gynecological Oncology, University of California Davis Medical Center, Sacramento, CA, United States.
Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, CA, United States.
Gynecol Oncol Rep. 2023 Sep 28;50:101283. doi: 10.1016/j.gore.2023.101283. eCollection 2023 Dec.
Clear cell carcinoma (CCC) of the vulva is extremely rare. We report a case of a 54-year-old woman who presented with a 5 cm mass of the mons pubis. She underwent needle biopsy demonstrating CCC. She then underwent radical vulvectomy with bilateral inguinofemoral lymph node dissection. Surgical pathology revealed CCC of the vulva with lymphovascular space invasion (LVSI) and metastatic carcinoma in 1/7 inguinal lymph nodes. The patient has a history of endometriosis, raising suspicion that her CCC could have arisen from endometriosis in the mons. She completed adjuvant treatment with cisplatin and concurrent external beam radiation therapy with radiographic evidence of complete response. However, short-interval imaging demonstrated multi-focal recurrence, which was confirmed with supraclavicular lymph node biopsy. She then completed 8 cycles carboplatin, paclitaxel, and biosimilar bevacizumab-bvzr with favorable response on imaging. She was continued on bevacizumab maintenance. She was later started on pembroluzimab for disease progression based on new mediastinal adenopathy and worsening retroperitoneal lymphadenopathy. She received eight cycles of pembrolizumab with ongoing disease progression before enrolling in hospice and discontinuing cancer-directed treatment. As described in the related literature which we summarize here, the majority of reported cases of vulvar CCC arise from endometriosis implants at the site of prior episiotomy or from the Bartholin's gland. This patient had clinical history of endometriosis; prior tissue sampling was not performed to support the diagnosis. Given the absence of data regarding this rare type of primary vulvar cancer, treatment of this patient's disease was based on existing data specific to squamous cell carcinoma of the vulva and extrapolated from treatment guidelines for CCC of the ovary and endometrium. Continued research is needed on this rare form of vulvar carcinoma to determine the risk factors, prognostic factors, and treatment recommendations specific to this disease.
外阴透明细胞癌（CCC）极为罕见。我们报告一例54岁女性，其阴阜出现一个5厘米的肿块。她接受了针吸活检，结果显示为CCC。随后她接受了根治性外阴切除术及双侧腹股沟股淋巴结清扫术。手术病理显示外阴CCC伴淋巴管血管间隙浸润（LVSI），且7个腹股沟淋巴结中有1个出现转移癌。该患者有子宫内膜异位症病史，这引发了怀疑，即她的CCC可能源于阴阜的子宫内膜异位症。她完成了顺铂辅助治疗以及同步外照射放疗，影像学显示完全缓解。然而，短时间内的影像学检查显示出现多灶性复发，经锁骨上淋巴结活检得以证实。随后她完成了8个周期的卡铂、紫杉醇和生物类似药贝伐单抗 - bvzr治疗，影像学显示有良好反应。她继续接受贝伐单抗维持治疗。后来，基于新出现的纵隔淋巴结肿大和腹膜后淋巴结病恶化，她开始接受帕博利珠单抗治疗疾病进展。在进入临终关怀并停止癌症针对性治疗之前，她接受了8个周期的帕博利珠单抗治疗，但疾病仍持续进展。正如我们在此总结的相关文献中所描述的，大多数已报道的外阴CCC病例源于既往会阴切开部位的子宫内膜异位种植灶或巴氏腺。该患者有子宫内膜异位症的临床病史；之前未进行组织采样以支持诊断。鉴于缺乏关于这种罕见的原发性外阴癌的数据，该患者疾病的治疗基于外阴鳞状细胞癌的现有数据，并从卵巢和子宫内膜CCC的治疗指南中推断而来。对于这种罕见的外阴癌形式，仍需要持续研究以确定特定于该疾病的风险因素、预后因素和治疗建议。
