Chiyoda Tatsuyuki, Ishikawa Mitsuya, Nakamura Masaru, Ogawa Mariko, Takamatsu Kiyoshi
Department of Obstetrics and Gynecology, Tokyo Dental College Ichikawa General Hospital, Chiba, Japan.
J Obstet Gynaecol Res. 2011 Dec;37(12):1856-9. doi: 10.1111/j.1447-0756.2011.01637.x. Epub 2011 Sep 15.
Epithelioid sarcoma of the vulva is an extremely rare and aggressive tumor. This tumor most commonly occurs on the labia majora in women of reproductive age. The molecular pathogenesis remains largely unknown. Only 20 cases of vulvar epithelioid sarcoma have been reported to date and more than half have had poor outcomes. We report a successfully treated case of vulvar epithelioid sarcoma in a 33-year-old woman. We performed a radical vulvectomy with flap reconstruction. Three years after surgery, the patient remains well, showing no evidence of recurrence. Early tissue diagnosis of vulvar epithelioid sarcoma is essential because this tumor can be clinically misdiagnosed as a benign lesion. Gynecologists should be aware of this rare tumor variant and carefully plan the treatment.
外阴上皮样肉瘤是一种极其罕见且侵袭性强的肿瘤。这种肿瘤最常发生于育龄期女性的大阴唇。其分子发病机制在很大程度上仍不清楚。迄今为止，仅报道了20例外阴上皮样肉瘤病例，其中半数以上预后不良。我们报告了一例33岁女性外阴上皮样肉瘤成功治疗的病例。我们进行了根治性外阴切除术并采用皮瓣重建。术后三年，患者情况良好，无复发迹象。外阴上皮样肉瘤的早期组织诊断至关重要，因为该肿瘤在临床上可能被误诊为良性病变。妇科医生应了解这种罕见的肿瘤变体并仔细规划治疗方案。
