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Pheochromocytomas arising from outside the adrenal glands are called paragangliomas and constitute a rare disease and can occur in the pelvic retroperitoneum. Symptoms of excess catecholamine production, as well as elevated urine vanillylmandelic acid levels and serum and urine norepinephrine levels, are highly diagnostic for paraganglioma. Imaging can be helpful for the diagnostic of these pelvic tumors, differentiating them from other pelvic masses. We hereby present a case of pelvic malignant paraganglioma.
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