Upper cervical nerve sheath tumors (NST) arising mainly from C2 root and to lesser extent from C1 root are not uncommon, they constitute approximately 5-12% of spinal nerve sheath tumors and 18-30% of all cervical nerve sheath tumors, unique in presentation and their relationship to neighbouring structures owing to the discrete anatomy at the upper cervical-craniovertebral region, and have atendency for growth reaching large-sized tumors before manifesting clinically due to the capacious spinal canal at this region; accordingly the surgical approaches to such tumors are modified. The aim of this paper is to discuss the surgical strategies for upper cervical nerve sheath tumors. Eleven patients (8 male and 3 females), age range 28-63 years, with C2 root nerve sheath tumors were operated upon based on their anatomical relations to the spinal cord. The magnetic resonance imaging findings were utilized to determine the surgical approach. The tumors had extra- and intradural components in 10 patients, while in one the tumor was purely intradural. The operative approaches included varied from extreme lateral transcondylar approach(n = 1) to laminectomy, whether complete(n = 3) a or hemilaminectomy(n = 7), with partial facetectomy(n = 7), and with suboccipital craniectomy(n = 2). The clinical picture ranged from spasticity (n = 8, 72,72 %), tingling and numbness below neck (n = 6, 54,54 %), weakness (n = 6, 54,54 %), posterior column involvement (n = 4, 26,36 %), and neck pain (n = 4, 36,36 %). The duration of symptoms ranged from 1 to 54 months, total excision was performed in 7 patients; while in 3 patients an extraspinal component, and in 1 patient a small intradural component, were left in situ. Eight patients showed improvement of myelopathy; 2 patients maintained their grades. One poor-grade patient was deteriorated. The surgical approaches for the C2 root nerve sheath tumors should be tailored according to the relationship to the spinal cord, determined by magnetic resonance imaging.
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