Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.
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.
Topsakal
C
C
Department of Neurosurgery, Firat University, School of Medicine, Elazig, Turkey. cdtopsakal@yahoo.com
Akdemir
I
I
Tiftikci
M
M
Ozercan
I
I
Aydin
Y
Y
D000328:Adult; D014408:Biomarkers, Tumor; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local; D009442:Neurilemmoma / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D018318:Neurofibroma, Plexiform / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000473:pathology*; D018319:Neurofibrosarcoma / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D012584:Sciatic Nerve / Q000473:pathology* / Q000601:surgery; D013125:Spinal Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000601:surgery
D014408:Biomarkers, Tumor