We report a rare case of leiomyosarcoma of the inferior vena cava (IVC) in which F-18 fluorodeoxyglucose (F-18 FDG) positron emission tomography-computed tomography (PET-CT) scan provided vital evidence, which led to its diagnosis, in a background of procoagulant state of the patient, where previous ultrasound-Doppler and echocardiography studies were nonspecific and revealed bilateral lower limb deep vein thrombosis with thrombus in IVC. The whole body F-18 FDG PET-CT scan was done in view of no significant improvement in clinical status of the patient over few months in spite of appropriate medical management. FDG PET-CT scan revealed high grade uptake in a large mass lesion occupying the right atrium, extending superiorly into terminal superior vena cava, inferiorly into dilated IVC and probably into hepatic veins.
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