We report FDG PET/CT findings of fumarate hydratase-deficient renal cell carcinoma coincidence with para-aortic paraganglioma in a 27-year-old man. He presented with painless gross hematuria for 3 days. CT identified an enhancing lesion in the left kidney, suggestive of a tumor. 18F-FDG PET/CT imaging showed the mass with high FDG uptake. An adjacent FDG-avid nodule was also noted. Postoperative pathology supported a diagnosis of fumarate hydratase-deficient renal cell carcinoma of the left kidney mass, and the adjacent nodule was paraganglioma.
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