Multiple bilateral and symmetric C1-2 ganglioneuromas: A case report

BACKGROUND: Ganglioneuromas are rare tumors of the sympathetic nervous system that originate from neural crest sympathogonia. Since the cervical spine has rarely been reported as a site for ganglioneuroma, we present a case report on this uncommon manifestation. CASE SUMMARY: A 34-year-old male presented with a 4-month history of progressive paralysis of both upper limbs along with an unsteady gait. The touch sensitivity of both hands was reduced, and there was conspicuously high muscle tonus in his upper and lower limbs, along with hyperactive physiological reflection and deep reflexes. Magnetic resonance imaging showed several nodules around the C2-7 intervertebral foramena, among which the masses lying between C1 and C2 were obviously bilaterally compressing the spinal cord. Successful posterior decompression was performed without fixation and the tumors in the upper cervical spine were removed intact, with rapid relief of symptoms. The pathological diagnosis was ganglioneuroma. CONCLUSION: Multiple and bilateral ganglioneuroams are a rare occurrence in the cervical spine. In this case report, timely resection of the neoplasms around C1 and C2 resulted in spinal cord decompression, with rapid relief of symptoms and a good prognosis. Including the current case, we are aware of only seven such cases in the literature, of which four arose from Japan, one from China, and one from Spain. We suppose that ethnicity and geographic associations with this rare disease presentation may be an aspect for future consideration and investigation.