Primary retroperitoneal leiomyosarcoma: a clinical and pathological study of 11 cases

目的 探讨腹膜后平滑肌肉瘤患者的临床病理特点及治疗方法.方法 回顾性分析1994年11月至2014年8月我院收治的11例腹膜后平滑肌肉瘤患者的临床资料.男5例,女6例.年龄13～75岁,中位年龄50岁.以血尿、腹部包块、腹部疼痛等为主要表现.CT平扫可见肿物呈软组织密度,内部密度不均,增强后肿物不同程度强化.手术标本行常规HE染色及免疫组化染色检查.结合患者临床特征及影像学表现,对腹膜后平滑肌肉瘤的临床病理特征进行分析.结果 11例患者均行手术治疗.术后病理示肿瘤直径中位数为14.4 cm.病理均提示出血坏死,镜下均呈梭形细胞排列,细胞密集,细胞核有异型,核分裂象易见.术后化疗2例,放疗1例.随访3例,分别于术后2个月、3年、5年复发.结论 腹膜后平滑肌肉瘤致死率高、复发率高,临床与CT表现均无特异性,确诊仍需依靠病理组织学与免疫组化检查.Objective To analyze the clinical characteristics,pathology,CT characteristics and targeted therapy of primary retroperitoneal leiomyosarcoma treated at our institution during the past 20 years.Methods A retrospective review was performed among 11 patients of this disease.The main performances were hematuria,abdominal mass and abdominal pain.Non-enhanced CT scanning showed soft tissue density in all cases.Tri-phase enhanced scanning showed reinforcement in different degrees.Pathology results showed tissue necrosis.The pathology featured by routine HE and immumohistochemical staining.Results Surgical treatment was performed in all 11 patients.Postoperative pathology showed that median tumor diameter was 14.40cm.Spindle cell,cellular abnormity and mitotic figures could be seen through light microscope.During follow-up,3 cases recurrenced after 2 months,3 years and 5 years respectively.Conclusions Primary retroperitoneal leiomyosarcoma might have a high fatality rate and high recurrence rate.The clinical and CT features are unapparent.Pathology diagnosis is reliable,surgery is necessary to prolong life and cure disease.中文核心期刊要目总览(PKU)中国科技核心期刊(ISTIC)中国科学引文数据库(CSCD)12916-9193