Cervical Intramedullary Schwannoma with Syrinx: Case Report and Review of the Literature

Abstract

Schwannoma is a nerve sheath tumor originating from the Schwann cell. It is benign in nature and it arises from anywhere where Schwann cells can be found. It is rarely found in the parenchyma of the spinal cord. Intramedullary schwannomas (or neurilemmomas) without evidence of neurofibromatosis are rare spinal cord tumors. Intramedullary schwannoma was first reported in 1932 by Penfield. Our patient presented with neck pain, gradually worsening, weakness in the right upper and lower limbs, numbness in both shoulders, and a decrease in the grasping strength of both hands over a 4-year period. A magnetic resonance imaging of the spine showed a heterogeneously enhancing mass in the cervical spinal cord extending from the C2 to T1 levels with associated hemorrhagic changes. Histologically, the tumor was found to be composed of bland spindle cells with blunt-ended and sometimes wavy nuclei admixed with hyalinized vasculature. Surrounding reactive spinal cord parenchyma with frequent Rosenthal fibers was also observed. Focal Verocay bodies were evident, and with immunohistochemistry, there was diffuse and strong positivity for S100, which is confirmatory for the diagnosis of schwannoma. We report a case of cervical intramedullary schwannoma presented with syringobulbia in a young adult.

Keywords

• intramedullary schwannomas
• syrinx
• spinal cord tumor
• cervical