Ⅰ型神经纤维瘤病合并腹膜后恶性外周神经鞘瘤1例报告并文献复习

Type Ⅰ Neurofibromatosis With Retroperitoneal Malignant Peripheral Nerve Sheath Tumor: Case Report and Literature Review

本文报道2016年6月我科对1例既往有Ⅰ型神经纤维瘤病的25岁男性患者行腹腔镜下腹膜后肿物切除术。术中因肿瘤底部较深且与骶骨、盆壁粘连较紧,中转为开放手术。手术时间185 min。术中出血1500 ml,输注悬浮红细胞400 ml。术后第2天拔除尿管,第7天拔除盆腔引流管,第8天出院。术后病理诊断为腹膜后恶性外周神经鞘瘤。术后随访1个月,左侧足面麻木感,左下肢肌力Ⅲ级,排便、排尿正常,未见肿瘤复发或转移。我们认为手术应尽可能完全切除恶性外周神经鞘瘤,如果肿瘤侵犯神经,常需要把受累神经一并切除,但可能会造成相应的功能障碍。

This study reported one 25-year-old man with previous history of type Ⅰneurofibromatosis undergoing laparoscopic resection of abdominal mass in June 2016. Due to the tight adhesion between the bottom of tumor and sacral and pelvic wall,laparoscopic surgery was conversed to open surgery. The operation time was 185 min. During the operation,the blood loss was 1500 ml,and 400 ml of suspended red blood cells were infused. The catheter was removed 2 days after operation and the pelvic drainage tube was removed 7 days after operation. After 8 days the patient was discharged from hospital. The pathological results confirmed a retroperitoneal malignant peripheral nerve sheath tumor. Postoperative follow-up for 1 month found that the patient＇s left foot surface was numbness,and the left lower extremity muscle strength was grade Ⅲ. The defecation and urination were normal. No tumor recurrence or metastasis was detected. We deem that laparoscopic or open surgery,which should be as complete as possible,is the preferred method of treatment. The nerve often needs to be removed if involved,but this may cause the corresponding dysfunction.