源自神经纤维瘤的盆腔骶尾部恶性外周神经鞘瘤1 例报道①.pdfVIP

- 290 - 中国康复理论与实践2013 年3 月第19 卷第3 期 Chin J Rehabil Theory Pract, Mar. 2013, Vol. 19, No.3 DOI : 10.3969/j.issn. 1006-9771.2013.03.026 ·临床观察· 源于神经纤维瘤的盆腔骶尾部恶性外周神经鞘瘤1 例报道① 任大江，李放，张志成，关凯，时述山 [摘要] 目的 探讨源于神经纤维瘤的盆腔骶尾部恶性外周神经鞘瘤的临床特征与治疗。方法 报道1例患者的症状、体征、 影像学检查、手术、化疗及预后，并进行文献回顾。结果和结论 源于神经纤维瘤病恶性外周神经鞘瘤早期诊断治疗对患者具有 重要意义。肿瘤侵袭性强，治疗困难，预后不佳。 [关键词] 神经纤维瘤病；恶性外周神经鞘瘤；治疗；预后 Malignant Peripheral Nerve Sheath Tumors in the Pelvis and Sacrococcyx Originated from Neurofibromatosis: A Case Report and Literatures Review REN Da-jiang, LI Fang, ZHANG Zhi-cheng, et al. Orthopaedic Department, Beijing Military Area General Hospital, Beijing 100700, China Abstract: Objective To observe the characteristics and treatment of malignant peripheral nerve sheath tumor originated from neurofi- bromatosis and the outcome of the patients. Methods A case was reported and the related literatures were reviewed. Results and Conclu- sion Malignant peripheral nerve sheath tumor is a rare soft tissue malignancy, which is with highly invasion. There are some difficulties with the diagnosis and treatment, and the outcome is poor. Early diagnosis is very important for treatment. Key words: malignant peripheral nerve sheath tumor; neurofibromatosis; treatment; outcome [中图分类号] R730.264 [文献标识码] A [文章编号] 1006-9771(2013)03-0290-04 [本文著录格式] 任大江，李放，张志成，等. 源于神经纤维瘤的盆腔骶尾部恶性外周神经鞘瘤1例报道[J]. 中国康复理论与实 践, 2013, 19(3): 290-293. 恶性外周神经鞘瘤(malignant peripheral nerve 患者消瘦，跛行，全身多发牛奶咖啡斑，大小不 sheath tumor, MPNST) 是一种非常罕见的软组织肿 一，大者直径4 cm 。全身多处可触及大小不等皮下结 瘤，多来源于周围神经或显示向神经鞘膜分化的恶性 节，质软，直径约2.5 cm 。无视力下降。L ~S 棘突间 5 1 肿瘤，也可以由良性的神经纤维瘤恶变而来。该肿瘤 隙压痛，无下方放散。左侧髂脊后方可扪及直径约6 临床表现和影像学缺乏特异性，诊断存在一定困难； cm 包块，