Etiology and Outcomes of Pediatric Spinal Cord Tumors

Abstract

Spinal cord tumors are a rare entity in the pediatric population. Pediatric oncology patients may develop spinal cord compression from tumors in the extradural, intradural extramedullary or intramedullary space. There are a variety of genetic syndromes that have a predisposition for spinal cord tumors, most notably Neurofibromatosis Type 2. Most spinal cord tumors tend to be slow growing with insidious presentation. Common symptoms may consist of pain, weakness, sensory changes, sphincter dysfunction and spinal deformity. The pattern of involvement depends upon tumor location in the cord. Because symptoms are often vague, slowly progressive and difficult to assess in young children, there is often a significant delay between symptom onset and diagnosis.

The most important predictors of overall survival (OS) and progression free survival (PFS) are the histologic grade and the degree of resection or treatment response. OS and PFS are often very good for most tumor types as low-grade histology predominates. The best predictor of neurologic outcome in virtually any tumor type is the degree and duration of neurologic impairment prior to treatment. Neurologic deficit and progressive spinal deformity are common. Overall function tends to improve over time with a combination of neurologic recovery and learning compensatory skills. Rehabilitation is an important part of the patient’s care to support maximum function and quality of life. The importance of maintaining functional independence and begins in the early stages and continues as long as there is neurologic deficit. Long-term morbidities as well as risk of tumor recurrence necessitates long term follow up both for screening and support.