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Chordoma Mimicking the Imaging Appearance of a Nerve Sheath Tumor Extending Through the Neural Foramen – a Case Report of 2 Patients 2014

Category General Spine Ashish R. Gandhe, MD
Jawaad Mohiuddin
Tripti Chopade
Adam Flanders, MD
Purpose We report two rare cases of chordoma presenting as an extradural mass exiting through an expanded neural foramen and mimicking the nerve sheath tumor. To our knowledge, about ten cases of lateral chordomas expanding the neural foramen have been published. Preoperative correct diagnosis is important, as radical resection of chordoma is essential to reduce the risk of recurrence. Materials & Methods Case 1: A 24-year-old lady presented with neck pain, numbness, tingling, and mild weakness in hands. MRI of the cervical spine showed a T1 hypointense, T2 hyperintense enhancing extradural mass, extending via an expanded right C4-C5 neural foramen into paraspinal soft tissues and encasing the vertebral artery. The mass was displacing the spinal cord posteriorly with associated cord signal abnormality. CT scan of the cervical spine confirmed erosions of C4-C5 vertebral bodies and pedicles. Imaging favored a neurofibroma. In view of bone erosions, possibilities of malignant degeneration of neurofibroma or lymphoma were also considered. CT guided biopsy reported chordoma. The patient underwent en bloc resection including the right vertebral artery. Case 2: A 52 year old lady presented with neck and left shoulder pain, tingling and numbness in left arm for two months. MRI of the cervical spine demonstrated a large T1 hypointense, T2 hyperintense, lobulated extradural mass at C4-C5, extending via an enlarged right neural foramen into the paraspinal soft tissues and displacing the vertebral artery laterally. The mass was abutting the spinal cord with no cord compression or signal abnormality. CTA of the neck confirmed patent right vertebral artery and revealed erosions of the C5 vertebra. Imaging favored nerve sheath tumor and other neoplasms were considered unlikely. The patient underwent en bloc resection with preservation of the vertebral artery. Histopathology confirmed the diagnosis of chordoma. The patient subsequently received radiation. 1.pptx Results Chordomas are low grade malignant osseous tumors arising from remnants of the embryonic notochord. Chordomas are generally midline with the ends of the neuraxis (sacrococcygeal and spheno-occipital regions) being favoured locations. Lateral location of a chordoma is extremely rare with only 10 reported cases of chordomas involving the neural foramina. Conclusion The imaging findings of chordoma involving the neural foramen mimic nerve sheath tumor. Neurofibroma is the most common tumor involving the neural foramen, however generally is not associated with osseous erosions. In absence of neurofibromatosis, aggressive appearance of neural foraminal lesion should suggest the possibility of other neoplasms including chordomas. Preoperative diagnosis is critical as chordomas warrant complete resection, to prevent recurrence. References Boriani S, Bandiera S, Biagini R, Bacchini P, Boriani L, Cappuccio M, et al. Chordoma of the mobile spine: fifty years of experience. Spine 2006;4:493–503.