Malignant rhabdoid tumors of the spine are rare pediatric neoplasms that have a poor prognosis. The histological, ultrastructural, and immunohistochemical features are essential elements used in their diagnosis. We report the case of a malignant rhabdoid tumor of the cervical spine in a 13-month-old infant. Tumor cells were vimentin positive with prominent nucleoli indented by eosinophilic cytoplasmic inclusions containing intermediate filaments. INI1 immunostaining was negative. This patient was operated on twice for symptomatic spinal cord compression. Despite chemotherapy, she developed worsening leptomeningeal dissemination, lower cranial nerve dysfunction, and hydrocephalus that did not respond to CSF diversion. She died 4 months after initial diagnosis. We review the literature on spinal malignant rhabdoid tumor and discuss the nomenclature, pathology, radiology, treatment, and outcomes of this rare entity.
Copyright 2009 S. Karger AG, Basel.