STUDY DESIGN: Case report. OBJECTIVE: To discuss the spinal presentation and treatment of paraganglioma, a rare tumor of the extra-adrenal paraganglia, derived from neural crest cells in sympathetic ganglions. SUMMARY OF BACKGROUND DATA: Malignant paragangliomas are a very rare entity. The diagnosis of malignant paraganglioma is made by local recurrence after total resection of the primary mass, or findings of distant metastases. Roughly half of distant metastases are located in the cervical lymph nodes, and the remainder is evenly distributed among bone, lung, and liver. Vertebral metastases are exceedingly rare, and only isolated case reports have described them. METHODS: A 71-year-old man reported increasing neck pain and upper extremity weakness. The patient had a previous carotid body tumor resection in the past and a contralateral carotid body tumor left intact. Our imaging workup, including plain radiograph, computerized tomography scan and MRI, revealed an intraosseous lesion at C6 with vertebral body destruction. The patient underwent decompressive surgery through an anterior Smith-Robinson approach for myelopathy, followed by adjuvant external beam radiation. The histologic and immunohistochemical assays revealed a malignant paraganglioma. RESULTS: Paragangliomas are often highly vascular tumors. Intraoperative blood loss was significant and may have been mitigated by preoperative embolization. The patient showed clinical improvement almost immediately after surgery. CONCLUSION: We report a rare case of malignant paraganglioma and discussed adjuvant treatments to consider for distant metastases, such as I-MIBG, conventional radiotherapy, and chemotherapy.
STUDY DESIGN: Case report. OBJECTIVE: To discuss the spinal presentation and treatment of paraganglioma, a rare tumor of the extra-adrenal paraganglia, derived from neural crest cells in sympathetic ganglions. SUMMARY OF BACKGROUND DATA: Malignant paragangliomas are a very rare entity. The diagnosis of malignant paraganglioma is made by local recurrence after total resection of the primary mass, or findings of distant metastases. Roughly half of distant metastases are located in the cervical lymph nodes, and the remainder is evenly distributed among bone, lung, and liver. Vertebral metastases are exceedingly rare, and only isolated case reports have described them. METHODS: A 71-year-old man reported increasing neck pain and upper extremity weakness. The patient had a previous carotid body tumor resection in the past and a contralateral carotid body tumor left intact. Our imaging workup, including plain radiograph, computerized tomography scan and MRI, revealed an intraosseous lesion at C6 with vertebral body destruction. The patient underwent decompressive surgery through an anterior Smith-Robinson approach for myelopathy, followed by adjuvant external beam radiation. The histologic and immunohistochemical assays revealed a malignant paraganglioma. RESULTS:Paragangliomas are often highly vascular tumors. Intraoperative blood loss was significant and may have been mitigated by preoperative embolization. The patient showed clinical improvement almost immediately after surgery. CONCLUSION: We report a rare case of malignant paraganglioma and discussed adjuvant treatments to consider for distant metastases, such as I-MIBG, conventional radiotherapy, and chemotherapy.