Object
This study is a retrospective analysis of 60 surgically treated patients with 64 peripheral nerve sheath tumors (PNSTs) at the second cervical (C-2) nerve root. The anatomical subtleties of these tumors and their implications for surgical strategy when compared with other spinal PNSTs and other tumors in the foramen magnum region are reviewed.
Methods
Sixty patients with C-2 PNSTs treated surgically in the Department of Neurosurgery at King Edward VII Memorial Hospital and Seth Gordhandas Sunderdas Medical College between 1992 and 2006 were studied. All patients underwent magnetic resonance imaging. Tumors were divided into 3 groups depending on their anatomical location identified during surgery. Those tumors located within or extending into the spinal dural tube were called Type A, those located within the dural tube of the C-2 ganglion were labeled as Type B, and tumors extending laterally into the paraspinal region were labeled as Type C. Follow-up durations ranged from 6 months to 15 years (mean 64 months).
Results
There were 38 male and 22 female patients in the study, who ranged in age from 6 to 62 years (mean 28 years). Nine patients had clinical features indicative of neurofibromatosis (NF). The mean duration of symptoms at the time of presentation was 27 months (range 4 days–5 years). Two patients had no specific symptoms related to the C-2 PNST, 6 patients had only local symptoms such as neck pain or stiffness, and 52 patients had symptoms of varying degrees of myelopathy. There were 5 solely Type A tumors, 7 Type A + B tumors, 31 Type B tumors, and 21 Type B + C tumors. All Type A, A + B, and B tumors were totally resected. Seven of 21 Type B + C tumors were partially resected, and the remainder were completely resected. All patients postoperatively reported varying improvement in their preoperative symptoms. Except for patients with NF who were disabled by other tumors, the rest of the patients resumed their normal life style. There have been no cases of symptomatic tumor recurrence.
Conclusions
The majority of PNSTs located at the C-2 level in these patients probably arose from the large C-2 ganglion and are limited within the dural confines or are interdural in location. In contrast to other spinal PNSTs, the location of C-2 PNSTs is in most cases posterior to the lateral mass of the atlas and axis and the atlantoaxial joint and is exposed to the posterior without any bone cover. Radical tumor resection is safe, resolution of clinical symptoms is rapid, and recurrence rates are extremely low. In a selected number of cases, bone work for tumor exposure and resection can be entirely avoided.
Treatment of malignant peripheral nerve sheath tumors in pediatric NF1 disease
