Malignant peripheral nerve sheath spinal tumours are relatively rare.
A primary spinal location at onset from the nerve roots is rarely repo
rted in the literature, thus the clinical features and therapeutic res
ults of these spinal malignant tumours are not defined. Six cases of m
alignant primary spinal schwannomas, 2% of 293 spinal schwannomas oper
ated on in a 38 year period, are reported. Based on an analysis of a l
imited number of cases, ours and those collected from the literature (
21 patients), some suggestions are possible: a) pre-operative clinical
presentation and imaging studies are not predictive of malignancy; b)
postoperative outcome is poor, especially in patients with von Reckli
nghausen's disease and after partial removal of the tumour; c) local r
ecurrence and metastases are possible, even after radical surgery and
radiotherapy.