Rodero L, Canga A, Figols J, Berciano J, Combarros O
Servicio de Neurologia, Hospital Universitario Marqués de Valdecilla, Universidad de Cantabria, Santander, Spain.
Neurologia. 2004 Jan-Feb;19(1):27-31.
Malignant peripheral nerve sheath tumors (MPNST) are rare. We report a case of a MPNST of the proximal sciatic nerve in the thigh, unassociated with pre-existing type 1 neurofibromatosis or history of radiation therapy. A 71-year-old man had a 6 month history of constant, severe, burning pain affecting the sole of the left foot. One month after the onset, the pain radiated to the left calf, posterior aspect of the thigh and buttock, and distal leg weakness followed. Three months prior to admission, the patient developed a large and painful mass in the buttock, that occupied the entire left gluteal region on examination. There was severe weakness of ankle and toe dorsiflexion and plantarflexion, decreased sensation on the lateral and posterior aspect of the left leg as well as on the dorsal and plantar surfaces of the foot, and absent ankle jerk. EMG showed denervation and motor unit loss in the short head of biceps femoris and muscles supplied by tibial and peroneal nerves on the left side. Magnetic resonance imaging revealed a 10-cm enhancing mass of the left sciatic nerve from the upper thigh to the greater sciatic notch. In surgery, a large MPNST with a high Ki67 labeling index (> 60 %) was subtotally removed from the sciatic nerve, and adjuvant radiation therapy was administered. In the ensuing months the tumor invaded the entire pelvic region. A high sciatic malignant tumor can present with a rapidly growing buttock mass and unilateral, neuropathic foot pain imitating the tarsal tunnel syndrome.
恶性外周神经鞘瘤(MPNST)较为罕见。我们报告一例发生于大腿近端坐骨神经的MPNST病例,该病例与既往存在的1型神经纤维瘤病或放射治疗史无关。一名71岁男性有6个月持续严重灼痛病史,疼痛累及左脚底。发病1个月后,疼痛放射至左小腿、大腿后侧和臀部,随后出现小腿远端无力。入院前3个月,患者臀部出现一个大的疼痛性肿块,检查发现肿块占据整个左臀区域。踝关节和足趾背屈及跖屈严重无力,左小腿外侧和后侧以及足背和足底感觉减退,跟腱反射消失。肌电图显示左侧股二头肌短头以及胫神经和腓总神经支配的肌肉出现失神经和运动单位丧失。磁共振成像显示从大腿上部至坐骨大切迹的左侧坐骨神经有一个10厘米的强化肿块。手术中,从坐骨神经上大部切除了一个Ki67标记指数较高(>60%)的大MPNST,并给予辅助放疗。在随后的几个月里,肿瘤侵犯了整个盆腔区域。高位坐骨恶性肿瘤可表现为臀部肿块迅速增大以及单侧神经性足部疼痛,类似跗管综合征。