Spinal intradural primary malignant peripheral nerve sheath tumor with leptomeningeal seeding: case report and literature review

involvement of the cervical spinal cord and diffuse leptomeningeal metastasis.We also reviewed the literature to discuss the management strategies and prognosis of primary spinal MPNST.We performed the systematic search by two researchers independently conducting the initial identification, and abstract screening.The eligible studies were discussed and the disagreements were resolved by means of discussion.The search was conducted on March 2015 in MedLine and EMBASE databases.For primary identification of articles, we searched the phrase "[(malignant) AND (peripher*) AND (nerv*) AND (tumor)]" setting no limitations, and if the abstract was not available, full text of article was used for screening evaluation.We assessed all studies regarding "the malignant peripheral nervous sheath tumors" located or involving the spinal canal. █ INTRODUCTION Malignant peripheral nerve sheath tumor (MPNST) is a sarcoma arising from Schwann cells and neural crest cells (20).They constitute 5% of all soft tissue sarcomas with the incidence of 0.001% (10), and a peak incidence in the seventh decade of life (11); occurring more in patients with neurofibromatosis I (NF-1) (13).MPNSTs are usually located in extremities, trunk, and head and neck regions in order of decreasing frequency (19).Primary spinal intradural MPNSTs are extremely rare (1,17,22,23), with frequent association with NF-1 (9).Here, we presented an exceedingly rare case of MPNST diagnosed in a 3-year-old girl, with intradural extra-medullary 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.