Summary
We present a 25-year-old male patient with neurofibromatosis type 1 (NF1). Thoracic intra-dural extra-medullary tumoral mass was excised gross-totally and the patient was referred to oncology unit. Histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST), a rare sarcoma with a dismal prognosis. Tumor recurred in its previous site with an adjacent apical mass in the left lung 7 weeks following initial surgery and repeat surgery was performed with complete removal of intra-dural tumor. We report the first patient with intra-dural MPNST localized proximal to conus medullaris; in upper thoracic spine. It must always be considered the possibility of a rare but a devastating tumor, MPNST beside schwannomas and neurofibromas in patients with NF1 when an intra-spinal mass is diagnosed.
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Albayrak, B.S., Gorgulu, A. & Kose, T. A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1. J Neurooncol 78, 187–190 (2006). https://doi.org/10.1007/s11060-005-9086-9
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DOI: https://doi.org/10.1007/s11060-005-9086-9