Nadkarni T D, Rekate H L, Coons S W
Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona, USA.
J Neurosurg. 1999 Jul;91(1 Suppl):112-5. doi: 10.3171/spi.1999.91.1.0112.
Plexiform neurofibroma of the cauda equina has been reported only twice previously. The authors report the first pediatric patient in whom such a tumor has been found. A 4-year-old boy presented with low-back pain that radiated bilaterally into the L-4 and L-5 dermatomes. A dermal sinus noted at the midthoracic level was surrounded by a hemangiomatous lesion. Magnetic resonance imaging confirmed the presence of the dermal sinus and revealed a well-defined lumbosacral mass that showed heterogeneous intensity with irregular enhancement. Intraoperatively, a solid mass, which engulfed the entire cauda equina, could not be dissected from the roots. The dermal sinus tract, however, was excised from the thoracic spine. The patient underwent radiotherapy to control the tumor and relieve his pain. Plexiform neurofibromas of the cauda equina are characterized by an insidious and progressive clinical course. The tumor mass may engulf all the roots of the cauda equina. No plexiform neurofibroma of the cauda equina has been reported to be associated with neurofibromatosis Type 1. The authors assume that the thoracic-level dermal sinus observed in this child was an incidental finding.
马尾神经丛状神经纤维瘤此前仅被报道过两次。作者报告了首例发现此类肿瘤的儿科患者。一名4岁男孩出现下背部疼痛，疼痛双侧放射至L-4和L-5皮节。在胸段发现一个皮窦，周围有一个血管瘤样病变。磁共振成像证实了皮窦的存在，并显示出一个边界清晰的腰骶部肿块，其信号强度不均匀，强化不规则。术中发现一个实性肿块，包裹了整个马尾神经，无法从神经根上分离。然而，皮窦道从胸椎处切除。患者接受了放疗以控制肿瘤并缓解疼痛。马尾神经丛状神经纤维瘤的特点是临床病程隐匿且呈进行性发展。肿瘤肿块可能会包裹马尾神经的所有神经根。此前尚无报道称马尾神经丛状神经纤维瘤与1型神经纤维瘤病相关。作者认为在该患儿中观察到的胸段皮窦是一个偶然发现。
