Ulbright T M, Brokaw S A, Stehman F B, Roth L M
Cancer. 1983 Oct 15;52(8):1462-9. doi: 10.1002/1097-0142(19831015)52:8<1462::aid-cncr2820520821>3.0.co;2-y.
The authors describe two cases of epithelioid sarcoma of the vulva and summarize the clinical and pathologic experience with this vulvar neoplasm. Findings indicate that epithelioid sarcoma of the vulva usually occurs in the labia majora of young women, but may occur in later life. Clinically it may mimic a Bartholin's duct cyst, thus leading to inadequate treatment. Pathologic findings are characterized by sheets and nests of acidophilic, polygonal cells. Follow-up study suggests that vulvar epithelioid sarcoma behaves more aggressively than extragenital epithelioid sarcoma. Four of five patients died of metastatic sarcoma. The courses of three patients were quite rapid, whereas a fourth patient had multiple recurrences over a long period, which is typical of extragenital epithelioid sarcoma. Complete initial excision is important. All patients with local recurrence died with distant metastases. Vascular invasion also indicates a poor outcome.
作者描述了两例外阴上皮样肉瘤病例，并总结了关于这种外阴肿瘤的临床和病理经验。研究结果表明，外阴上皮样肉瘤通常发生于年轻女性的大阴唇，但也可能在较晚年龄段出现。临床上，它可能类似巴氏腺囊肿，从而导致治疗不充分。病理表现为嗜酸性多边形细胞的片状和巢状结构。随访研究表明，外阴上皮样肉瘤比生殖器外上皮样肉瘤的侵袭性更强。五名患者中有四名死于转移性肉瘤。三名患者的病程进展相当迅速，而第四名患者在很长一段时间内多次复发，这是生殖器外上皮样肉瘤的典型表现。初次完整切除很重要。所有局部复发的患者均死于远处转移。血管侵犯也提示预后不良。
