Spinal intradural malignant peripheral nerve sheath tumor at the foramen magnum in a non-neurofibromatosis child.

Abstract

Primary spinal intradural malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, especially in children with a non-neurofibromatosis background. Scarce pediatric data exist with regard to such tumors. A 4-year-old child with a history of spastic limb weakness was operated for a foramen magnum spinal lesion (intradural and extradural) with imaging suggestive of schwannoma; the histopathology, however, was that of a MPNST. He underwent redo-surgery for recurrent infiltrating lesion, became ventilator dependent, and expired. Along with this case, we briefly discuss the relevant literature on pediatric primary spinal MPNSTs. The report represents an unusual site for spinal MPNST in a child with dual components. It is important for clinicians to consider such malignant lesions among the differentials of paediatric spinal intradural/ extradural lesions. Compared to that of adults and other regions, the prognosis for spinal intradural MPNSTs in children remains grim.