外阴上皮样肉瘤的临床分析

目的 探讨外阴上皮样肉瘤的临床特点、诊断治疗方法及预后.方法 对我院收治的4例和文献报道资料完整的16例外阴上皮样肉瘤患者进行回顾性分析.结果 20例外阴上皮样肉瘤患者年龄23～80岁,中位年龄36岁.肿物直径为1～10 cm,中位数为5.1 cm.免疫组化检测结果显示,20例外阴上皮样肉瘤患者vimentin阳性,19例CK阳性,16例EMA阳性.10例患者行双侧或同侧腹股沟淋巴结清扫,3例未行腹股沟淋巴结清扫.术后7例患者未补充治疗,13例患者行术后放疗和(或)化疗.20例外阴上皮样肉瘤患者中,无复发及转移11例(55.0%),腹股沟淋巴结转移治疗后存活2例(10.0%),死亡7例(35.0%).早期患者(Ⅰ～Ⅱ期)的中位生存时间(21个月)高于晚期患者(6个月,P＜0.05).10例行双侧或同侧腹股沟淋巴结清扫患者的中位生存时间为11.5个月,3例未行腹股沟淋巴结清扫患者的中位生存时间为6个月,差异无统计学意义(P=0.086).结论 外阴上皮样肉瘤误诊率较高,确诊时需要结合免疫组化检查,诊断上皮样肉瘤的标记性蛋白为vimentin、EMA和CK.外阴上皮样肉瘤的有效治疗方法为局部广泛切除加双侧腹股沟淋巴结清扫,手术切缘至少2 cm,化疗和放疗的疗效尚不确切.

Clinical characterization of vulvar epithelioid sarcoma

Objective Vulvar epithelioid sarcoma is a rare, undifferentiated soft-tissue sarcoma,with a high rate of local relapse, regional nodal spread and distant metastases. The aim of this study was to investigate the clinical features, diagnosis, treatment and prognosis of this malignancy. Methods We studied the clinicopathologic features of 20 cases of vulvar epithelioid sarcoma, of which 4 cases were admitted to our hospital from 1999 to 2009. All of the patients received radical local excision with inguinofemoral lymphadenectomy. Seven patients were treated without adjuvant therapy. Seven patients received postoperative radiotherapy only and three underwent chemotherapy. Chemotherapy plus radiotherapy were given postoperatively in three. Results The patients ranged in age from 23 to 80 years(median: 36y). The tumors ranged from 1 to 10 cm in their greatest diameter(median: 5. 1 cm). All cases showed immunoreactivity for both vimentin and cytokeratin. Follow-up information on all 20 patients was available,and covered periods ranging from 3 to 104 months. 11 patients were alive with no evidence of disease.2 patients developed lymph node metastases but alive. 7 patients had died of the disease. Survival of the early stage(Ⅰ - Ⅱ)patients was significantly longer than those in the advanced stage(Ⅲ-Ⅳ)(median,21vs. 6 months, P ＜0.01). There was no significant difference between survival of patients with or without ingninofemoral lymphadenectomy(median, 11.5 vs. 6 months, P = 0.086). Conclusions Because of the relatively frequent misdiagnosis, a differential diagnosis combined with immunohistochemistry is needed to determine an early and accurate diagnosis. The tumor markers exhibiting immunoreactivity includ vimentin,epithelial membrane antigen(EM A)and cytokeratin(CK). Radical local excision with adequate margin(at least 2 cm)and bilateral inguinofemoral lymphadenectomy is effective for the treatment of vulvar epithelioid sarcoma. The role of adjuvant therapy, chemotherapy and radiation remains unclear but merits consideration.