Atypical teratoid rhabdoid tumor of lumbar spine in a child, masquerading as ependymoma

Atypical teratoid/rhabdoid tumors (ATRT) are rare embryonal tumors of the central nervous system system (CNS) which predominantly occur in children less than three years of age and accounts for 1–2% of all pediatric CNS malignancies, most commonly in intracranial locations. The spinal cord is a rare primary site of ATRT with a prevalence of only 2% of all CNS ATRT, with few case reports. We report the case of an 8-yeat-old child initially thought to have lumbar spinal ependymoma, based on clinical presentation and imaging. However careful neuropathology review, with comprehensive immuno-histochemistry, confirmed the diagnosis of spinal ATRT. The child was treated with radiation therapy and adjuvant chemotherapy, and continues to do well.