原发性外阴尤因肉瘤/原始神经外胚层肿瘤：1例报告并文献复习
Primary vulvar Ewing sarcoma/primitive neuroectodermal tumor: a report of one case and review of the literature.
作者信息
Yang Jie, Guo Qi, Yang Yunpeng, Zhang Junji, Lang Jinghe, Shi Honghui
机构信息
Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China.
出版信息
J Pediatr Adolesc Gynecol. 2012 Aug;25(4):e93-7. doi: 10.1016/j.jpag.2012.03.005.
BACKGROUND
Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are aggressive malignancies characterized by translocation involving the EWS gene. Primary extraskeletal ES/PNET rarely affects the female genital tract.
CASE
We report the case of a 20-year-old female, who presented with a rapidly growing painful mass arising from the left labium majus for six months. Multiple lesions in bones and nodules in lungs were considered metastasis. The diagnosis was established by immunohistochemical stain and reverse transcriptase polymerase chain reaction analysis. The patient ceased treatment and died of respiratory failure one month after discharge.
CONCLUSION
Vulvar ES/PNET mainly affects adolescent females. ES/PNET should be considered in the differential diagnosis of tumors involving the lower gynecologic tract in adolescent female.
背景
尤因肉瘤/原始神经外胚层肿瘤（ES/PNET）是一种侵袭性恶性肿瘤，其特征是涉及EWS基因的易位。原发性骨外ES/PNET很少累及女性生殖道。
病例
我们报告一例20岁女性患者，其左侧大阴唇出现一个迅速生长的疼痛性肿块，持续6个月。骨骼中的多个病灶和肺部结节被认为是转移灶。通过免疫组织化学染色和逆转录酶聚合酶链反应分析确诊。患者停止治疗，出院后1个月死于呼吸衰竭。
结论
外阴ES/PNET主要影响青春期女性。在青春期女性下生殖道肿瘤的鉴别诊断中应考虑ES/PNET。
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