腹膜后恶性外周神经鞘瘤的临床特征及外科诊治分析  

Clinical Features and Surgical Diagnosis and Treatment of Retroperitoneal Malignant Peripheral Nerve Sheath Tumor

作　　者：唐茂盛 肖萌萌 陈小兵 邹博远 李文杰 罗成华[1] Tang Maosheng;Xiao Mengmeng;Chen Xiaobing(Department of Retroperitoneal Tumors,Peking University International Hospital,Beijing 102206,China)

机构地区：[1]北京大学国际医院腹膜后肿瘤外科,北京102206

出　　处：《中国微创外科杂志》2022年第12期966-970,共5页Chinese Journal of Minimally Invasive Surgery

摘　　要：目的探讨腹膜后恶性外周神经鞘瘤的临床特征及外科诊治疗效。方法回顾性分析2015年4月~2021年10月16例腹膜后恶性外周神经鞘瘤的临床资料。散发性12例,继发于Ⅰ型神经纤维瘤病4例。肿瘤单发13例,多发3例。肿瘤最大径5~30 cm,(16.53±7.12)cm,其中13例≥10 cm。5例包绕重要血管。均行手术切除。结果完整切除10例,姑息性切除6例。联合脏器切除6例。分块切除5例。术中出血量100~6000 ml,中位数2000 ml。术后并发症6例,死亡1例。16例中位生存期24个月。完整切除10例中位生存期25.0月,姑息性切除6例中位生存期14.0月。结论腹膜后恶性外周神经鞘瘤恶性程度高,侵袭性强,手术治疗困难。Objective To explore the clinical features and surgical efficacy for retroperitoneal malignant peripheral nerve sheath tumors.Methods Clinical data of 16 cases of retroperitoneal malignant peripheral nerve sheath tumor from April 2015 to October 2021 were retrospectively analyzed.There were 12 cases of sporadic disease and 4 cases secondary to typeⅠneurofibromatosis.There were 13 cases of single tumor and 3 cases of multiple tumors.The maximum diameter of tumor was 5-30 cm(mean,16.53±7.12 cm),13 of which were≥10 cm.The tumor surrounded important blood vessels in 5 cases.All the patients underwent surgical resection.Results There were 10 cases of complete resection and 6 cases of palliative resection.There were 6 cases of combined organ resection.The tumor was resected in blocks in 5 cases.The intraoperative blood loss was 100-6000 ml(median,2000 ml).There were 6 cases of postoperative complications.One patient died.The median survival of the 16 cases was 24 months,which was 25.0 months in the 10 patients with complete resection and 14.0 months in the 6 patients with palliative resection.Conclusion Retroperitoneal malignant peripheral nerve sheath tumors are highly malignant and aggressive,which are difficult to treat surgically.

关 键 词：恶性外周神经鞘瘤 腹膜后肿瘤 

分 类 号：R735.5[医药卫生—肿瘤]