A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites including the head, neck, thorax and abdomen). Unlike other types of cancer, there is no test that determines benign from malignant tumors; long–term followup is therefore recommended for all individuals with paraganglioma. Approximately 50% of patients with recurrent disease experience distant metastasis. The five–year survival in the setting of metastatic disease is 40% to 45%.