ABSTRACT
Paragangliomas are benign neoplasms that arise from the autonomic nervous system and the associated paraganglia. Although benign, they have been shown to possess metastatic potential. Involvement of the spine is rare. Even rarer is considered the involvement of the cervical spine. We report a case of a patient with a history of an extra-adrenal non-functional paraganglioma of the jugular foramen which was initially treated with intra-arterial embolization. After a 3-year disease-free follow-up, the patient was presented with symptoms of spinal cord compression due to spinal metastases in C2 and C3 vertebrae. The patient was then treated with surgical decompression and external beam radiation. Therapeutic management with additional treatment options is now under discussion by a multidisciplinary team. Paraganglioma of the jugular foramen with spinal metastasis is an uncommon presentation where increased physician awareness and long-term follow-up are mandatory for all patients with history of paraganglioma.
ABSTRACT
Paragangliomas are benign neoplasms that arise from the autonomic nervous system and the associated paraganglia. Although benign, they have been shown to possess metastatic potential. Extra-adrenal retroperitoneal paraganglioma with vertebral metastasis is considered very uncommon. Here, we present a case of a functional extra-adrenal paraganglioma of the retroperitoneum giving metastasis to T4 vertebra after five years of follow-up in a 48-year-old man who had been initially treated with complete resection of the primary tumor. The condition of the patient improved significantly after radiosurgery and somatostatin analogs treatment, until lumbar spine lesions appeared six months later. Our case demonstrates that retroperitoneal paraganglioma is a rare condition which should be considered in the differential diagnosis of a retroperitoneal mass combined with vertebral lesions. Additionally, increased physician awareness and long-term follow-up is mandatory for all patients with history of retroperitoneal paraganglioma since metastases may occur after long latent intervals from the initial diagnosis.