腹膜后平滑肌肉瘤的临床诊治特点分析(附26 例报告)  

Diagnosis and treatment of retroperitoneal leiomyosarcoma(report of 26 cases)

作　　者：陈菁华 陈启晨 赵建军 毕新宇 赵宏 李智宇 蔡建强 周健国 Chen Jinghua;Chen Qichen;Zhao Jianjun;Bi Xinyu;Zhao Hong;Li Zhiyu;Cai Jianqiang;Zhou Jianguo(Department of Hepatobiliary Surgery,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肝胆外科,北京100021

出　　处：《中国医刊》2021年第5期492-496,共5页Chinese Journal of Medicine

摘　　要：目的探讨腹膜后平滑肌肉瘤的临床特点及治疗方法。方法回顾性分析2005年1月至2020年1月中国医学科学院北京协和医学院肿瘤医院收治的26例腹膜后平滑肌肉瘤患者的临床资料,总结其临床特点、治疗方法及预后情况。结果26例中男4例,女22例,确诊年龄27~83岁(中位年龄51岁)。以腹痛、腹胀、腹部包块等为主要表现,CT检查可见肿瘤形态不规则,呈分叶状,病灶内部密度不均,可呈不同程度强化,且囊变和坏死较多。所有患者均行手术治疗,完整切除21例,姑息性切除5例。术后病理示肿瘤最大径直径3.5~30.0cm(中位数9.9cm),肿瘤组织主要由梭形细胞组成,呈轻中度异型性,可见核分裂象,免疫组化示SMA、Vimentin、Desmin阳性。术后化疗13例,放疗9例。18例患者病情进展,其中13例为远处转移,5例为局部复发。总生存时间7~182个月(中位时间26个月),1、3、5年生存率分别为96.0%、50.0%、40.0%。结论腹膜后平滑肌肉瘤临床与CT表现均无特异性,确诊需依靠病理组织学及免疫组化检查,手术完整切除是首选的治疗方式,辅助治疗的有效性尚未明确,需进一步探究。Objective To investigate the clinical features,diagnosis,treatment and prognosis of retroperitoneal leiomyosarcoma.Method The clinical data of 26 patients with retroperitoneal leiomyosarcoma admitted to Cancer Hospital Chinese Academy of Medical Sciences from January 2005 to January 2020 were retrospectively analyzed,and the clinical features,treatment and prognosis of the patients were summarized.Result There were 4 males and 22 females.The median age of diagnosis was 51 years(range 27-83 years).The main performances were abdominal pain,bloating,and abdominal masses.CT scans showed that the tumor was irregular in shape and lobulated and the tumor was reinforced to different degrees after enhancement.All patients underwent surgical treatment,and 21 underwent macroscopically complete resection.Postoperative pathology showed that the median maximum diameter of the tumor was 9.9 cm(3.5-30.0 cm).The tumor tissue was mainly composed of spindle cells,with mild to moderate cellular atypia and mitotic figures.Immunohistochemistry represented positive SMA,Vimentin,and Desmin.Thirteen cases received chemotherapy and 9 cases received radiotherapy.The disease progressed in 18 cases.Thirteen cases had distant metastasis,and the remaining 5 cases had local recurrence.The median survival time was 26 months(7-182 months),and the 1,3,and 5-year survival rates were 96.0%,50.0%and 40.0%,respectively.Conclusion The clinical and imaging characteristics of retroperitoneal leiomyosarcoma are non-specific.The diagnosis depends on histopathological and immunohistochemical examination.Macroscopically complete resection remains the most curable treatment,and the effectiveness of adjuvant therapy needs to be further explored.

关 键 词：腹膜后肿瘤 平滑肌肉瘤 手术治疗 辅助治疗 

分 类 号：R738.7[医药卫生—肿瘤]