Case report of an acute presentation of diplegia in a four-month old - to expect the unexpected!

Spinal tumors are rare in infancy. Among them, atypical teratoid/rhabdoid tumor of the spine is an even more rare pediatric neoplasm with grave prognosis. Here we present a case of atypical teratoid/rhabdoid tumor (ATRT) of the lumbar spine in a four months old male who presented with acute onset of lower limb weakness. Magnetic resonance imaging of the spinal cord revealed a highly cellular intradural intramedullary embryonal tumour from D8-L4. Immunohistochemically, integrase inhibitor 1(INI-1) stain show loss of expression. Despite surgical resection, the infant presented with new metastatic lesions and hydrocephalus. Ventriculo-peritoneal shunt was placed and the prognosis was explained to the parents.

Keywords: Intramedullary, Rhabdoid tumor, Hydrocephalous.