Yu Dongwoo, Choi Joon Hyuk, Jeon Ikchan
Department of Neurosurgery, Yeungnam University Hospital, Yeungnam University College of Medicine, 170, Hyeonchung street, Nam-Gu, Daegu, 42415, South Korea.
Department of Pathology, Yeungnam University Hospital, Yeungnam University College of Medicine, Daegu, South Korea.
BMC Musculoskelet Disord. 2020 Jul 11;21(1):454. doi: 10.1186/s12891-020-03492-y.
Plexiform schwannoma (PS), variant of schwannoma, often involves multiple fascicles as plexiform neurofibroma, and is usually located superficially on the dermis and subcutaneous layers. Spinal PS is extremely rare, and there is insufficient information on its natural course and treatment strategy. We describe the clinical features and treatment of giant intradural PS at the lumbosacral spine.
A 66-year-old man presented with leg pain, paresthesia, and weakness for 2 years. Magnetic resonance imaging demonstrated a large mass lesion involving a continuous multi-lobulated bead-like mass and a cystic portion from L1 to S3. The lesion was iso-intense on T2-weighted images (WI), iso- to slightly low-intense on T1-WI, and heterogeneous enhancement on contrast-enhanced T1-WI. The large mass lesion had three portions, including a cystic mass at L1, continuous multi-lobulated bead-like mass with a cystic portion from L2 to S1, and multi-lobulated mass from S2 to S3, which were identified with severe adhesions with cauda equina on operative assessment. Grossly total extirpation was achieved at the lumbar spine, and remained three round shaped small masses at the lumbar area and a multi-lobulated round masses from S2 to S3 involving nerves related with motor function of the lower extremities and anal sphincter, respectively. Histological examination revealed multinodular or plexiform growth pattern composed of spindle-shaped tumor cells, which were diffusely and strongly positive for S100 protein with KI67 < 1%. There were no recurrence of preoperative symptoms and changes of the remained masses over a 2-year follow-up period.
Subtotal extirpation to minimize neural deficits and close observation can be considered an appropriate treatment strategy for a giant spinal PS considering its benign prognosis and histological features, with a high risk of neurological damage during surgery.
丛状神经鞘瘤（PS）是神经鞘瘤的一种变体，常像丛状神经纤维瘤一样累及多个束状结构，通常位于真皮和皮下层浅表部位。脊柱PS极为罕见，关于其自然病程和治疗策略的信息不足。我们描述了腰骶部巨大硬膜内PS的临床特征及治疗情况。
一名66岁男性因腿部疼痛、感觉异常和无力症状就诊，病程2年。磁共振成像显示一个巨大肿块病变，为连续的多叶状串珠样肿块及从L1至S3的囊性部分。该病变在T2加权像（WI）上呈等信号，在T1加权像上呈等至稍低信号，在增强T1加权像上呈不均匀强化。这个巨大肿块病变有三个部分，包括L1处的囊性肿块、从L2至S1的连续多叶状串珠样肿块及囊性部分、从S2至S3的多叶状肿块，手术评估发现其与马尾神经严重粘连。在腰椎部位实现了大体全切，但在腰部区域仍残留三个圆形小肿块，以及从S2至S3的一个多叶状圆形肿块，分别累及与下肢运动功能和肛门括约肌相关的神经。组织学检查显示由梭形肿瘤细胞组成的多结节或丛状生长模式，S100蛋白弥漫性强阳性，KI67＜1%。在2年的随访期内，术前症状未复发，残留肿块也无变化。
考虑到其良性预后和组织学特征，以及手术中神经损伤风险高，对于巨大脊柱PS，可考虑行次全切除以尽量减少神经功能缺损并密切观察，这可能是一种合适的治疗策略。
