Paragangliomas are rare neuroendocrine tumors that can vary in size and metabolic activity. We report a case of giant bilateral malignant retroperitoneal paragangliomas (PGL) in a patient with germline succinate dehydrogenase B (SDHB) mutation. This patient, who presented in an emaciated and debilitated state, was managed with adrenergic blockade followed by radical primary surgery. After being metabolically and radiographically disease free for 4 years, he underwent salvage resection for recurrent retroperitoneal disease and palliative radiation to a site of solidary vertebral metastasis. We review incidence and prognosis of metastatic PGL.
Keywords: Giant paraganglioma; Paraganglioma, (PGL); Retroperitoneal; Succinate dehydrogenase B subunit, (SDHB); Succinate dehydrogenase mutation; Succinate dehydrogenase, (SDH).
Published by Elsevier Inc.