A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1.
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.
Albayrak
Baki S
BS
Department of Neurosurgery, Suleyman Demirel University, Medical Faculty Hospital, Isparta, Turkey. serdarbaki@gmail.com
Gorgulu
Askin
A
Kose
Turgay
T
D000328:Adult; D004388:Dura Mater / Q000473:pathology; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000601:surgery / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000150:complications / Q000473:pathology*; D013120:Spinal Cord Neoplasms / Q000473:pathology* / Q000601:surgery / Q000628:therapy; D013904:Thoracic Vertebrae; D016896:Treatment Outcome