PDF(Pubmed)
Abstract：
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.
摘要：
外阴透明细胞癌（CCC）极为罕见。我们报告了一例54岁的女性，她的阴户质量为5厘米。她接受了穿刺活检显示CCC。然后，她接受了根治性外阴切除术，并进行了双侧腹股沟股淋巴结清扫。手术病理显示外阴CCC伴淋巴管间隙侵犯（LVSI），腹股沟1/7淋巴结转移癌。患者有子宫内膜异位症病史,令人怀疑她的CCC可能是由于蒙斯的子宫内膜异位症引起的。她完成了顺铂辅助治疗和同时进行的外部束放射治疗，并有影像学证据表明完全缓解。然而,短间隔成像显示多灶性复发，经锁骨上淋巴结活检证实。然后她完成了8个周期的卡铂，紫杉醇,和生物相似物贝伐单抗-bvzr在影像学上有良好的反应。她继续接受贝伐单抗维持治疗。后来，她开始使用pembroluzimab治疗新的纵隔淋巴结肿大和腹膜后淋巴结肿大恶化的疾病进展。在接受临终关怀并停止癌症定向治疗之前，她接受了八个周期的pembrolizumab，疾病进展持续。正如我们在这里总结的相关文献中所描述的那样，报道的外阴CCC的大多数病例来自于先前会阴切开术部位的子宫内膜异位症植入物或Bartholin腺体。该患者具有子宫内膜异位症的临床病史；先前未进行组织取样以支持诊断。鉴于缺乏关于这种罕见类型的原发性外阴癌的数据，该患者疾病的治疗是基于现有的针对外阴鳞状细胞癌的数据，并根据卵巢和子宫内膜CCC的治疗指南推断。需要继续研究这种罕见的外阴癌，以确定危险因素，预后因素，以及针对这种疾病的治疗建议。
