The authors report a rare case of primary disseminated intradural malignant peripheral nerve sheath tumor (MPNST) of the spine in a 5-year-old child without neurofibromatosis type I (NF-I). This child presented with abdominal pain and gait disturbance. MRI revealed a large intradural extramedullary tumor at C4-5 with dissemination to the thoracic spine, cauda equina and leptomeninges. Following a 2-level cervical laminectomy, the tumor was biopsied and debulked. Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST. Because of the rarity of this pathological entity, a review of the literature was performed. The reported clinical outcomes for adult and pediatric patients with intradural MPNST are very poor. No established standard for the treatment of these tumors exists. We report the first pediatric case--without or with NF-I--of disseminated intradural MPNST primarily localized proximal to the conus medullaris. It must always be considered in the differential diagnosis for intradural extramedullary tumors of the pediatric spine, along with neurofibromas and schwannomas, even in children without NF-I.
Copyright 2009 S. Karger AG, Basel.