原发性腹膜后平滑肌肉瘤31例诊治分析

Analysis of diagnosis and treatment of 31 cases of primary retroperitoneal leiomyosarcoma

目的探讨原发性腹膜后平滑肌肉瘤的临床特点、诊治及预后。方法回顾性分析2010年1月至2018年6月华中科技大学同济医学院附属协和医院诊治的原发性腹膜后平滑肌肉瘤病人的临床资料。结果共纳入31例病人,其中男性9例,女性22例,确诊中位年龄58岁(25~80岁)。临床首发症状包括腹痛及腹部不适13例,体检偶然发现7例,腰痛5例,下肢肿胀2例,腹部包块2例,血尿1例,便血1例。肿瘤中位直径为8 cm(3~25 cm)。31例病人均行手术治疗,28例(90.3%)肿瘤完整切除,其中10例(35.7%)行联合脏器完整切除。中位随访时间56个月,随访28例,19例(67.9%)出现复发转移,其中8例(28.6%)复发转移后死亡。复发转移病人中12例接受再次手术治疗,复发后中位总生存期为24个月;7例未接受再次手术治疗,复发后中位总生存期为13个月。结论手术完整切除是原发性腹膜后平滑肌肉瘤的首选治疗方式;腹膜后平滑肌肉瘤术后复发率高,预后较差;对无明显手术禁忌的复发转移病人,在多学科团队的指导下应积极行再次手术治疗。

Objective To explore the clinical features,diagnosis,treatment and prognosis of primary retroperitoneal leiomyosarcoma(PRLS).Methods The clinical data of patients with primary retroperitoneal leiomyosarcoma diagnosed and treated in Union Hospital,Tongji Medical College of Huazhong University of Science and Technology from January 2010 to June 2018 were analyzed retrospectively.Results A total of 31 patients were included,including 9 males and 22 females,with a median age of 58 years(range,25 to 80 years).The first clinical symptoms included abdominal pain and abdominal discomfort in 13 cases,incidental findings in 7 cases,lower back pain in 5 cases,lower limb swelling in 2 cases,abdominal mass in 2 cases,hematuria in 1 case,and hematochezia in 1 case.The median diameter of the tumor was 8(3-25)cm.Surgical treatment was performed in all 31 patients,among which 28 patients(90.3%)und-erwent complete resection,and 10 patients(35.7%)underwent combined visceral resection.After a median follow-up time of 56 months among 28 patients,19 patients(67.9%)developed recurrence and metastasis,of whom 8(28.6%)died after recurrence and metastasis.Among the patients with recurrence or metastasis,12 received reoperation and the median overall survival after recurrence was 24 months;7 patients did not receive reoperation and the median overall survival after recurrence was 13 months.Conclusion Complete surgical resection is the preferred treatment of PRLS.The postoperative recurrence rate of retroperitoneal leiomyosarcoma is high and the prognosis is poor.For patients with recurrence or metastasis without obvious surgical contraindications,reoperation should be actively performed under the guidance of multiple disciplinary team.