Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1 - Case report

被引:20
作者
Topsakal, C
Akdemir, I
Tiftikci, M
Ozercan, I
Aydin, Y
机构
[1] Firat Univ, Sch Med, Dept Neurosurg, Elazig, Turkey
[2] Firat Univ, Sch Med, Dept Pathol, Elazig, Turkey
[3] Sisli Etfal Hosp, Dept Neurosurg, Istanbul, Turkey
关键词
neurilemmoma; neurofibroma; malignant peripheral nerve sheath tumor; malignant schwannoma; von Recklinghausen's disease;
D O I
10.2176/nmc.41.551
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.
引用
收藏
页码:551 / 555
页数:5
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