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We report the case of 38-year-old male with a malignant and functional paraganglioma arising from the retroperitoneum. He was admitted to our hospital with a pathological fracture of the right femur, A diagnosis of paraganglioma was made after taking a biopsy specimen and discovering a hypervascular retroperitoneal tumor. The level of catecholamine in his urine was greatly increased, accompanied with hypertension, rapid pulse and sweating. He was treated with chemotherapy, radiotherapy and surgical therapy. However, he died from pulmonary metastasis.
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