腹膜后黏液纤维肉瘤的诊治

Diagnosis and treatment of retroperitoneal myxoifbrosarcoma

目的：分析腹膜后黏液纤维肉瘤的治疗方法及预后相关因素。方法对福建医科大学附属第一医院近年收治的确诊为腹膜后黏液纤维肉瘤的患者进行分析，总结其临床表现、手术治疗方案及预后相关因素。结果2005至2013年共有5例患者确诊为腹膜后黏液纤维肉瘤，临床表现主要为腰、腹痛。手术完整切除4例，预后良好，随访4年无复发。1例肿瘤残留，术后6个月复发。结论黏液纤维肉瘤缺乏特异性的临床症状、体征及影像学表现，确诊主要依赖组织病理学检查。手术切除不完整易导致术后复发，完整切除结合放化疗有助于改善患者预后。

ObjectiveTo investigate the clinical characteristics, treatment and prognosis of retroperitoneal myxofibrosarcoma.MethodsThe clinical data of a rare case of retroperitoneal myxofibrosarcoma was analyzed retrospectively and the related literature were reviewed.ResultsFive cases of retroperitoneal myxofibrosarcoma from 2005 to 2013 were found in our hospital databases. The general clinical presentation were lumbodynia and abdominal distention. 4 case received en-block resection of the tumor without recurrence and 1 case received partial resection with recurrence in the 6 months.Conclusion Myxofibrosarcoma is a kind of disease lack of typical symptoms, signs and imaging features. Its diagnosis is greatly based on histopathological examination. Easy to relapse, myxofibrosarcoma was treated by surgical resection combined with radiotherapy and chemotherapy, from which the prognosis of the patient improved.