Department of Gynecologic Oncology, Dr. Zekai Tahir Burak Women's Health Education and Research Hospital, Ankara, Turkey.
J Gynecol Oncol. 2011 Sep;22(3):211-3. doi: 10.3802/jgo.2011.22.3.211. Epub 2011 Sep 28.
Two types of gynecologic tumors are commonly described in the Turner syndrome, the first one is gonadoblastoma, which occurs in patients with Y chromosome abnormalities, and the second one is endometrial carcinoma which is mostly related with exogenous estrogen usage. Here, we describe an extremely rare case of squamous cell carcinoma of the vulva in a virgin woman with Turner syndrome. A 35-years old single, virgin woman referred to our Oncology Department with warty, necrotized, exophytic 6-7 cm vulvar mass. She had a history of primary amenorrhea and mosaic Turner syndrome was determined in her karyotype analysis. Biopsy specimen of the vulvar mass revealed squamous cell carcinoma of the vulva, and total vulvectomy with inguinal femoral lymphadenectomy was performed. The postoperative course was uneventful and there has been no recurrence of the disease up to date. Women with Turner syndrome have streak ovaries that produce very low estrogen and the squamous cell carcinoma of the vulva may have developed at an early age with Turner syndrome because of this low estrogen value similar to postmenopausal women. The current case is a special case due to its age of occurrence, virgin and Turner syndrome status.
特纳综合征中常描述两种妇科肿瘤，第一种是性腺母细胞瘤，发生于存在 Y 染色体异常的患者中，第二种是子宫内膜癌，其与外源性雌激素的使用关系密切。本文描述了一例特纳综合征处女患者发生外阴鳞癌的极其罕见病例。一位 35 岁的单身处女因外阴有疣状、坏死、外生性 6-7cm 肿块而被转诊至我院肿瘤科。她有原发性闭经病史，核型分析显示为嵌合体特纳综合征。外阴肿块的活检标本显示外阴鳞癌，行外阴广泛切除术和腹股沟股淋巴结切除术。术后恢复顺利，至今未发现疾病复发。特纳综合征患者的卵巢为条索状，产生的雌激素极低，由于这种低雌激素水平类似于绝经后妇女，因此外阴鳞癌可能在特纳综合征早期发生。本病例由于其发病年龄、处女和特纳综合征状态较为特殊。
