A Case of Cervical Chordoma

Abstract

A 48 year old male presented with neck pain and left arm pain that worsened with movement alongside left arm weakness, numbness, and tingling. The differential diagnosis for a patient with symptoms of a neck mass includes benign tumors like neurofibromas, malignant tumors like chordomas, and non-neoplastic conditions like cervical spondylitis. A magnetic resonance imaging (MRI) study of the cervical spine with and without contrast identified a T1 hypointense, T2 hyperintense, heterogeneously enhancing prevertebral mass with parapharyngeal extension. A direct laryngoscopy with biopsy was performed and revealed a paraspinal tumor. The patient’s diagnosis of cervical chordoma was confirmed upon detection of Brachyury, a gene that encodes a transcription factor which promotes epithelial mesenchymal transition (EMT) in chordoma pathogenesis. Chordomas are slow-growing tumors located within the body’s midline and they are associated with poorer outcomes because of neurovascular encasement at time of presentation. Chordomas are rare, with an incidence of 1 in 1,000,000. Approximately 6% of chordomas are located in the cervical spine. They are typically treated with surgery followed by radiation therapy. The patient underwent anterior resection for the prevertebral mass in the C2-C6 section of the cervical spinal cord. Surgery achieved subtotal resection and involved removal of the spinous processes of C3-C5 and reconstruction of the cervical spine with implants. The patient will be starting proton beam radiotherapy for his adjuvant treatment. Although rare, it is important to keep chordomas in the differential diagnosis when evaluating a patient with a neck mass.