Halil S, Kucuk M, Arvas M, Aydin O, Calay Z Z
Department of Obstetrics and Gynecology, Turkish Kidney Foundation, Hizmet Hospital, Istanbul, Turkey.
Eur J Gynaecol Oncol. 2011;32(1):117-8.
Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES-PNET) is a high-grade malignant neoplasm that often develops in the skeletal system. Primary extraskeletal ES-PNET is an uncommon condition that rarely affects the female genital tract. Tumors in the ovary, cervix, and uterine corpus and vulva are occasionally reported. Reports on the Ewing family of tumors involving the vulva are extremely rare in the relevant literature. Only a few cases of vulvar ES-PNET have so far been reported.
A 14-year-old adolescent girl presented to the clinic with a 4-month history of a left vulvar mass. The mass was excised under general anesthesia, and re-resection was performed three weeks later to obtain negative microscopic margins. The patient received chemotherapy and radiotherapy; however, she died of pulmonary metastasis within nine months of the initial surgery.
In summary, we describe a rare case of vulvar ES-PNET with distinct rosette-like structures in a 14-year-old adolescent girl with a very poor prognosis.
尤因肉瘤/外周原始神经外胚层肿瘤（ES-PNET）是一种高级别恶性肿瘤，常发生于骨骼系统。原发性骨外ES-PNET较为罕见，很少累及女性生殖道。卵巢、宫颈、子宫体和外阴的肿瘤偶尔有报道。有关文献中，关于尤因肿瘤累及外阴的报道极为罕见。迄今为止，仅报道了少数几例外阴ES-PNET病例。
一名14岁青春期女孩因左侧外阴肿物4个月就诊于诊所。肿物在全身麻醉下切除，三周后再次切除以获得阴性显微镜切缘。患者接受了化疗和放疗；然而，她在初次手术后九个月内死于肺转移。
总之，我们描述了一例罕见的外阴ES-PNET病例，该病例发生在一名14岁青春期女孩身上，具有独特的玫瑰花结样结构，预后极差。
