原发性腹膜后平滑肌肉瘤临床病理分析  

The Clinical and Pathological Analysis of Primary Retroperitioneal Leiomyosarcoma

作　　者：张竹青[1] 赵俊军[1] 李平[1] ZHANG Zhuqing;ZHAO Junjun;LI Ping(Department of Pathology,Affiliated Municipal Central Hospital of Dalian Medical University,Dalian City,Liaoning Province 116033)

机构地区：[1]大连医科大学附属中心医院病理科,辽宁省大连市116033

出　　处：《医学理论与实践》2021年第6期913-915,共3页The Journal of Medical Theory and Practice

摘　　要：目的:探讨原发性腹膜后平滑肌肉瘤(PRLS)的临床病理学特点、诊断治疗和预后。方法:收集2013年10月—2020年9月诊断的8例PRLS患者的临床病理资料。分析PRLS组织病理学表现及不同抗体的表达情况,随访患者生存及肿瘤复发情况。结果:8例PRLS中男1例,女7例,中位年龄51岁。大体:肿瘤平均直径8.7cm,切面灰白,部分伴出血、坏死及囊性变。镜下:肿瘤细胞呈梭形束状排列,有异型,可见核分裂和多核瘤巨细胞。免疫表型:Desim、SMA均为细胞质阳性表达。随访7例,其中2例术后复发、转移,分别于术后9个月、13个月因疾病进展死亡;1例为肿瘤晚期,无手术适应证,拒绝放化疗,首诊3个月后死亡;1例伴肝、肺及腹腔转移,术后规律化疗及靶向治疗,已随访48个月,疾病稳定;3例肿瘤完全切除,随访时间3~20个月,均无复发。结论:PRLS无特异性的临床表现与影像学特征,确诊需靠病理组织学及免疫组化检查,PRLS手术治疗后复发转移率高,预后较差。Objective:To analyze the clinical and pathological characteristics,differential diagnosis and prognosis of primary retroperitioneal leiomyosarcoma(PRLS).Methods:Clinical datas of 8 patients with PRLS from October 2013 to September 2020 were analyzed retrospectively.The pathomorphologic features and different antibodys were analyzed.These patients were followed up to detected the survival and tumor recurrence.Results:1 male and 7 female with an median age of 51 years were included.Gross pathology showed that median tumor diameter was 8.7cm,and the masses may be gray and white,some tumors with hemorrhage,necrosis,or cystic change.In histologically,these tumors showed spindle cells with nuclear atypia,mitoses and macromonoma giant cells.Immunohistochemical results showed that Desmin and SMA were positive in cytoplasm.7 patierts were followed up.2 patients died of tumor recurrence and metastasis at 9 months and 13 months respectively.1 patient was diagnosed as advanced tumor and died 3 months after the first diagnosis without surgical indication and chemotherapy and radiotherapy were refused.1 patient with liver,lung and abdominal metastasis received regular chemotherapy and targeted therapy after operation,and was stable for 48 months.3 patients whose tumors were completely resected were followed up for 3~20 months without recurrence.Conclusion:Without specific clinical and imaging manifestations,the accurate diagnosis of PRLS depends on pathological morphology and immunohistochemical analysis.PRLS has high recurrence and metastasis rate and poor prognosis.

关 键 词：腹膜后肿瘤 平滑肌肉瘤 免疫组织化学 

分 类 号：R738.6[医药卫生—肿瘤]