A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1


Albayrak B. S. , Gorgulu A., Kose T.

JOURNAL OF NEURO-ONCOLOGY, vol.78, no.2, pp.187-190, 2006 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 78 Issue: 2
  • Publication Date: 2006
  • Doi Number: 10.1007/s11060-005-9086-9
  • Title of Journal : JOURNAL OF NEURO-ONCOLOGY
  • Page Numbers: pp.187-190

Abstract

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.