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.
Highlights
Paragangliomas, known as extra-adrenal pheochromocytomas, are neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and from neuroendocrine cells of the extra-adrenal autonomic paraganglia, with the majority of the tumours being benign in nature [1]
It is believed that vertebral metastases with neural compression should be treated with decompressive surgery and total en bloc spondylectomy. He et al treated a similar case of metastatic retroperitoneal paraganglioma with resection of the vertebral metastasis and posterior fixation surgery
We report a case of a functional extra-adrenal paraganglioma of the retroperitoneum giving vertebral metastases five years after the complete removal of the primary site
Summary
Paragangliomas, known as extra-adrenal pheochromocytomas, are neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and from neuroendocrine cells of the extra-adrenal autonomic paraganglia, with the majority of the tumours being benign in nature [1]. There have been several reports of metastatic paraganglioma in the literature, but rarely do such tumors metastasize to the spine [2, 3]. In this case report, the patient was diagnosed with a metastatic lesion in T4 vertebra after five diseasefree years from the initial resection of a retroperitoneal paraganglioma. A 48-year-old man was admitted with the complaints of worsening back pain, palpitation, headache and sweating. He denied any trauma or weakness in his extremities. MRI revealed small new lesions in lumbar spine 6 months later (Figure 3 A,B,C). Treatment with combination chemotherapy for the management of the disease is under discussion by a multidisciplinary team
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