Malignant Retroperitoneal Paraganglioma in Adults

We report a case of a familial, malignant, retroperitoneal paraganglioma, because their low incidence,and especially in this case for their aggressive biological behavior with extensive metastatic and abdominal disease. Due to the fact that their histopathological diagnosis of malignancy is difficult, the characteristics of local infiltration or the distant metastases demonstrated by images specially for CT and RM are useful for their diagnosis. MIBG scintigraphic and PET (Positron Emission Tomography) with new radioisotopes, offer substantial sensitivity and specificity to correctly detect metastatic disease and helps identify patients suitable for treatment with radiopharmaceuticals. Must be considered the diagnosis of paraganglioma in the presence of a highly vascular mass located in anatomical areas where paraganglia exist in a patient with elevated catecholamines and normal adrenal glands.