|Spinal Intradural Primary malignant peripheral nerve sheath tumor with leptomeningeal seeding: case report and literature review|
|Humain Baharvahdat1, Babak Ganjeifar1, Nema Mohamadian Roshan2, Aslan Baradaran1|
|1Mashhad University of Medical Sciences, Neurological Surgery Department, Mashhad,
2Mashhad University of Medical Sciences, Pathology Department, Mashhad,
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 exam. 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 KaplanMeier 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.
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