Multifocal malignant peripheral nerve sheath tumor in patients with neurofibromatosis type I: Report of two cases and review of literature

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are one of the rarest soft-tissue sarcomas with a prevalence 0.001% in general population. It is closely associated unique neurocutaneous stigmata under spectrum dermatological manifestations neurofibromatosis type 1 (NF1). Almost 81% MPNST arises from precursor neuroma, and multifocality these lesions extremely rare, making up to cases. Moreover, spinal cases uncommon only four reported internationally. Here, we present fifth sixth brief review literature. Case Description: We describe two unusual multifocal relation NF1 occurring cord. Both patients presented local pain myelopathic symptoms. The underwent wide surgical resection, followed by neoadjuvant radiotherapy immediate postoperative improvement complaint; however, patient suffered rapid recurrence metastasis. Conclusion: Due scarcity related MPNST, no clear guidelines regarding management set Histopathological diagnosis remains as most pivotal diagnostic tool they can mimic other lesions, such neuromas schwannomas, imaging. Cases that were managed early intervention addition best outcome. However, concomitance found be resistant both chemo have high rate.