Spinal Intradural Primary Malignant Peripheral Nerve Sheath Tumor with Leptomeningeal Seeding: Case Report and Literature Review.

Abstract

Spinal intradural primary malignant peripheral nerve sheath tumors (MPNST) are rare in patients without neurofibromatosis. Here we represent a 3-year-old girl of primary intradural spinal malignant peripheral nerve sheath tumor. The tumor was removed partially and MPNST was diagnosed in the histopathological examination. Her condition deteriorated due to acute hydrocephalus in the following days. In this article, we discuss the clinical presentation, imaging, treatment, and prognosis of our patient and the other 22 patients of primary intradural MPNST, found in the literature. The Kaplan?Meier method was applied for univariate analysis and Cox proportional hazards model for multivariate analysis. This analysis showed that age, was an important factor predicting short-term survival of patients with MPNST.