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

被引:19
|
作者
Albayrak, Baki S. [1 ]
Gorgulu, Askin [1 ]
Kose, Turgay [1 ]
机构
[1] Suleyman Demirel Univ, Dept Neurosurg, Med Fac Hosp, Isparta, Turkey
关键词
intra-dural; neurofibromatosis type 1; malignant peripheral nerve sheath tumor;
D O I
10.1007/s11060-005-9086-9
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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.
引用
收藏
页码:187 / 190
页数:4
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