Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Taiwan J Obstet Gynecol. 2021 Jan;60(1):132-135. doi: 10.1016/j.tjog.2020.11.020.
To report a case of vulvar epithelioid sarcoma, a rare type of tumor that has been reported in 37 cases in the English literature to date.
We report three cases of vulvar epithelioid sarcoma, proximal type. Wide excisions of the mass were performed with margins free of tumor in all three cases.
Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. Locoregional lymph node involvement, vascular invasion, tumor size larger than 2 cm, deep localization, presence of necrosis, and a high mitotic index are known as poor prognostic factors. Adjuvant radiotherapy is advisable in the presence of a high-grade tumor or positive margins. The beneficial effect of adjuvant chemotherapy is not well established. Treatment decisions should be made based on the individual case presentation and pathology evaluation.
报告 1 例外阴上皮样肉瘤病例，这是一种罕见的肿瘤，迄今为止，英文文献中仅报道了 37 例。
我们报告了 3 例外阴上皮样肉瘤，近端型。所有 3 例均行肿块广泛切除术，切缘无肿瘤。
由于发病率低，目前尚无基于证据的诊断算法或治疗推荐。局部区域淋巴结受累、血管侵犯、肿瘤大小大于 2cm、深部定位、存在坏死和高有丝分裂指数是预后不良的因素。对于高级别肿瘤或切缘阳性，建议行辅助放疗。辅助化疗的有益效果尚未得到很好的确立。应根据个体病例表现和病理评估做出治疗决策。
