外阴近端型上皮样肉瘤临床病理特征及预后分析  

Clinicopathological characteristics and prognosis analysis of vulvar epithelioid sarcoma

作　　者：袁华 王亚希 李宁 吴令英 姚洪文 YUAN Hua;WANG Yaxi;LI Ning;WU Lingying;YAO Hongwen(National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100021,China)

机构地区：[1]国家癌症中心·国家肿瘤临床医学研究中心·中国医学科学院北京协和医学院肿瘤医院妇科,北京100021 [2]国家癌症中心·国家肿瘤临床医学研究中心·中国医学科学院北京协和医学院肿瘤医院病理科,北京100021

出　　处：《中华肿瘤防治杂志》2024年第22期1393-1398,共6页Chinese Journal of Cancer Prevention and Treatment

基　　金：中国医学科学院肿瘤医院-深圳医院合作基金(CFA202202018)。

摘　　要：目的回顾性分析外阴上皮样肉瘤(ES)患者的临床病理特征、治疗及预后情况,为临床诊治该类疾病提供参考。方法收集2003-01-01-2020-01-31中国医学科学院肿瘤医院经病理确诊并接受治疗的13例外阴ES患者临床资料,患者发病年龄28~65岁,中位发病年龄46岁。分析外阴ES患者发病年龄、临床表现、肿瘤大小、分期等。运用Kaplan-Meier法绘制生存曲线,计算生存率。结果根据国际妇产科协会(FIGO)2009分期标准,Ⅰ期8例(61.5%),Ⅱ期4例(30.8%),Ⅲ期1例(7.7%)。10例患者为本院初治,其中9例患者接受手术治疗,其中8例(8/9)患者接受外阴局部扩大切除术、1例(1/9)患者接受外阴广泛切除;8例(8/9)患者接受腹股沟淋巴结清除,1例(1/8)患者出现腹股沟淋巴结转移。5例(5/9)患者术后接受化疗,均未接受辅助放疗。中位随访时间为112.7个月(4.5~174.8)个月。10例初治患者5年和10年无进展生存率分别为67.5%和54.0%。5年和10年总生存率均为78.8%。共4例复发患者在我院治疗,均接受复发减瘤手术。结论外阴ES易发生于绝经前女性。大部分患者以触及外阴肿物就诊。根治性手术是主要治疗方式,辅助放化疗的作用仍需进一步探讨。Objective To analyze the clinical and pathological characteristics,treatment,and prognosis of patients with vul-var epithelioid sarcoma(ES),provide reference for the clinical diagnosis and treatment of this type of disease.Methods Clinical data of 13 cases of vulvar ES pathologically diagnosed and treated by pathology at the Cancer Hospital of the Chi-nese Academy of Medical Sciences from January 1,2003 to January 31,2020 were collected.The patients had an onset age of 28-65 years and a median onset age of 46 years.Such as age of onset,clinical manifestations,tumor size,and staging in patients with vulvar ES were analyzed.Kaplan Meier method was used to draw survival curve and calculate sur-vival rate.Results According to the 2009 staging criteria of the Federation International of Obstetrics and Gynecology(FIGO),there were 8 cases(61.5%)in stageⅠ,4 cases(30.8%)in stageⅡ,and 1 case(7.7%)in stageⅢ.Ten pa-tients were initially treated in our hospital,and 9 patients underwent surgical treatment.Among them,8 patients(8/9)underwent wide local excision,and 1 patient(1/9)underwent radical vulvectomy;Eight patients(8/9)underwent ingui-nal lymph node dissection,and one patient(1/8)had inguinal lymph node metastasis.Five patients(5/9)received chem-otherapy after surgery and did not receive adjuvant radiotherapy.The median follow-up time was 112.7(4.5-174.8)months.The 5-year and 10-year progression free survival rates of 10 newly treated patients were 67.5%and 54.0%,re-spectively.Both of the 5-year and 10-year overall survival rates were 78.8%,respectively.Four recurrent patients were treated in our hospital and underwent cytoreduction surgery.Conclusions Vulvar genital ES is more likely to occur in pre-menopausal women.Most patients are presented with vulvar mass.Radical surgery is the main treatment method,and adjuvant radiotherapy and chemotherapy still need further exploration.

关 键 词：外阴上皮样肉瘤 临床病理特征 预后 手术治疗 

分 类 号：R737.35[医药卫生—肿瘤]