Giant intradural plexiform schwannoma of the lumbosacral spine - a case report and literature review

BACKGROUND: 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. CASE PRESENTATION: 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. CONCLUSION: 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.