A 19-year-old woman presented with a primary mediastinal B-cell lymphoma invading the superior vena cava with associated thrombosis of the left brachiocephalic and subclavian vein. She underwent thrombolysis followed by chemotherapy. The midtreatment 18F-FDG PET/CT demonstrated important regression of the primary mediastinal B-cell lymphoma, but showed intense focal hepatic uptake in segment IV, without a corresponding lesion on ultrasonography, non–contrast-enhanced low-dose CT, and MRI. This focal uptake disappeared on a subsequent 18F-FDG PET/CT study when the radiotracer was injected in the foot, suggesting an anomalous venous return pathway that persisted despite thrombolysis.
lower extremity. 1,7 The passage of radiotracer from the lower extremity into the heart via inferior vena cava bypasses the obstructed superior counterpart, thereby avoiding the false-positive hepatic finding. 9 The presence of a genuine lesion is unlikely when delayed FDG PET images show changing configuration and/or reduced intensity of the focal hepatic FDG activity. It is postulated that the changing hemodynamics in the left portal venous system and/or the collaterals may account for the change on delayed images. Acquiring delayed images saves patients' time and additional radiation exposure for a repeat FDG PET study. In case of focal increased hepatic FDG activity near the falciform ligament in patients with SVCO, we propose that delayed acquisition of images should be attempted for further characterization of the activity before a repeat FDG PET is contemplated.
IntroductionPrimary cardiac paragangliomas are rare tumors. Metastatic disease is even rarer. Surgical management is technically challenging, and sometimes even impossible. Available therapeutic modalities for metastatic disease include external beam radiation therapy as well as systemic treatments, namely 131I-MIBG and more recently, peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE. To our knowledge, this is the first case of progressive unresectable cardiac paraganglioma with intracardiac extension treated with dosimetry based personalized PRRT to be reported. This case is of particular interest since it documents for the first time the efficacy, and especially the safety of the 177Lu-DOTATATE PRRT in this precarious context for which therapeutic options are limited.Case PresentationA 47-year-old man with no medical history consulted for rapidly decreasing exercise tolerance. The investigation demonstrated an unresectable progressing metastatic cardiac paraganglioma with intracardiac extension. The patient was treated with personalized 177Lu-DOTATATE PRRT and showed complete symptomatic and partial anatomical responses, with a progression-free survival of 13 months.ConclusionsPRRT with 177Lu-DOTATATE should be considered for inoperable cardiac paraganglioma. No major hemodynamic complications were experienced. Therapy resulted in safety and substantially improved quality of life.
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