Background: Paragangliomas of the head and neck are rare tumors. Moreover, malignant paragangliomas of the cervical spine are extremely rare. Currently, the combination of curative surgical resection and secondary adjuvant radiotherapy is the gold standard for treating symptomatic malignant paragangliomas. However, traditional treatments for malignant paraganglioma remain unsuccessful. The purpose of this study is to report an exceedingly rare case involving cervical metastasis of a malignant paraganglioma.
Case presentation: In this case report, we present a case involving a 72-year-old male with a history of paraganglioma of the neck. He had been experiencing bilateral shoulder pain, neck pain and weakness in the upper extremities for more than six months. Magnetic resonance imaging of the cervical spine revealed a metastasis at C4 with severe vertebral body destruction. To avoid serious complications associated with surgical resection, CyberKnife® radiosurgery (Accuray, Inc., Sunnyvale, CA, USA) was performed on the parapharyngeal and cervical lesions. A secondary surgery, which involved a posterior laminectomy at C3-6 and posterior fusion at C1-T1, was performed two weeks after the radiosurgery. A histological examination of the surgical specimen demonstrated a malignant paraganglioma. The patient regained strength in all extremities in the postoperative field, and his pain was dramatically reduced. A magnetic resonance imaging study performed three months after the surgery showed a reduced tumor size and spinal cord decompression.
Conclusion: This case study is the first report of a patient with symptomatic cervical metastasis of a malignant paraganglioma treated with a combination of radiosurgery and posterior spinal surgery. Although the optimal treatment for these conditions remains unclear, concomitant treatment with radiosurgery and reconstructive surgery appeared to be both safe and effective in this challenging case.