腹膜后平滑肌肉瘤的手术及临床病理特征

Clinical features and operative outcomes of retroperitoneal leiomyosarcoma

目的分析腹膜后平滑肌肉瘤(RPLMS)手术患者的临床结果及病理特征。方法回顾性分析2015年1月至2020年12月北京大学国际医院腹膜后肿瘤外科收治并手术的97例RPLMS患者的临床资料。结果97例患者中原发性RPLMS 49例,复发性或前次未完整切除者48例,最常见症状为腹痛、腹胀(30例)和腰背部疼痛(23例)。所有患者均接受RPLMS切除术,其中64例联合器官切除术,19例联合部分下腔静脉切除术,2例联合髂外动脉切除人工血管重建术,总体R0/R1切除率为84.5%,总体并发症发生率为26.8%,包括肠瘘4例,胰瘘1例,膀胱阴道瘘1例,尿瘘1例,胆瘘1例,腹腔内出血2例,下腔静脉、下肢静脉血栓7例,胃瘫3例等,其中1例腹腔大出血患者在术后30 d内死亡。术后病理提示梭形和多型性为最常见的病理亚型。结论RPLMS症状不典型,手术是主要治疗手段。联合多脏器、大血管的扩大切除,术后严重并发症发生率较高,应谨慎开展。

Objective To analyze the operative outcomes and postoperative pathological features of retroperitoneal leiomyosarcoma(RPLMS)undergoing surgeries.Methods Medical records of RPLMS patients admitted to Peking University International Hospital from Jan 2015 through Dec 2020 were retrospectively reviewed.Results Ninety-seven patients undergoing resectional surgeries were included in the study.Of whom,49 cases were primary RPLMS.Others were recurrent sarcomas or sarcomas with incomplete resection in the first surgical intentions.The most frequent symptoms were abdominal pain and distention(30 cases)as well as lower back pain(23 cases).All patients underwent resectional surgeries with a R0/R1 rate of 84.5%.Sixty-four cases received extended surgeries with combined organs resection.External iliac artery resection with reconstruction were performed on 2 cases.And 19 patients underwent partial IVC resection in combination of sarcoma resection.The general postoperative morbidity was 26.8%,including 4 intestinal fistulas,1 pancreatic fistula,1 vesicovaginal fistula,1 urinary fistula,1 biliary fistula,2 abdominal major bleeding,7 IVC thrombosis,3 gastroplegiaetc.One patient deceased within post-operative 30 d due to massive bleeding.Pathology found that spindle and pleomorphic cell types were most common subtypes of RPLMS.Conclusions Surgery remains the mainstay in the treatment of RPLMS which often presents with atypical symptoms.Extended surgeries combining with multiple organ and major vascular resections could be only suggested in experienced sarcoma centers due to high risk of severe postoperative complications.