Holocord Ependymoma in Association With Neurofibromatosis Type 2

A bstract An 11-year-old boy presented with a holocord intramedullary tumor, bilateral vestibular schwannomas, a right trigeminal schwannoma, a left suprasellar meningioma and two small meningiomas at the D5-D6 and D9-D10 vertebral levels. The clinical and radiological characteristics were suggestive of neurofibromatosis type 2. The intramedullary spinal tumor extended from the pontomedullary junction to the fifth dorsal vertebra level with an associated syrinx up to the conus. A single-stage near-complete excision of the tumor was performed, resulting in no added neurological deficits on long-term follow-up. The tumor histopathology was consistent with ependymoma. Although single-stage excision yields a favorable outcome, major challenges include the proximity to critical structures, long-segment involvement of the tumor, intraoperative blood loss, and later-stage spinal deformity. Intraoperative neuromonitoring and the use of an ultrasonic aspirator help achieve maximum safe resection of the tumor with minimal manipulation of the cord parenchyma. Laminoplasty is preferred to prevent spinal deformity at a later stage.