A 50-year-old female, known case of multinodular goiter, presented with chest discomfort, hemoptysis, and dyspnea to emergency. Electrocardiogram showed left bundle branch block. Hemoglobin was 8.8 g/dl. Chest X-ray showed multiple soft tissue opacities in both lung fields. Two-dimensional transthoracic echocardiography with color Doppler showed a mass of approximately 5.6 cm x 2.2 cm, attached to the right atrium [Figure 1] with mild pericardial effusion and global hypokinesia of left ventricle. The findings on echocardiography were of the opinion for primary cardiac myxoma. High-resolution computed tomography thorax showed multiple discrete soft tissue attenuated nodular lesions in peripheral lung fields with few irregular thick walled cavitatory lesions. The sputum and tuberculosis quantiferon test were negative for acid fast bacilli. Her serum thyroid stimulating hormone was 0.3 mIU/L. Ultrasound (USG) guided fine needle aspiration cytology (FNAC) from solid component of thyroid swelling was suggestive of colloid goiter.
The patient was subjected to FDG PET-CT whole body study for work up. PET-CT showed a well-defined heterogeneously enhancing hypodense mass in the right atrium with intense FDG uptake (SUVma x 19.2) along with pericardial infiltration and minimal pericardial effusion [Figure 2]. A non-FDG avid thrombus was seen in right superior pulmonary vein extending up to left atrium [Figure 3]. Multiple FDG avid parenchymal and subpleural nodules were seen in both lungs (SUVma x 3.0-12.5). The mediastinal and right hilar nodes were enlarged with increased FDG uptake (SUVma x 3.3-9.2). Liver showed multiple hypodense lesions in both lobes (SUVma x 4.0-12.2). Hypermetabolic ill-defined hypodense lesions were seen in head, uncinate process and body of pancreas (SUVma x 9.7). A well-defined heterogeneously enhancing lesion was seen in inter-polar region of the right kidney (SUVma x 3.6). Hypermetabolic enlarged peripancreatic (SUVma x 9.5) and aortocaval (SUVma x 10.5) nodes were also seen. A peripherally enhancing hypodense lesion measuring 1.5 cm x 1.4 cm with minimal perilesional edema and no significant FDG uptake was seen in left frontal cortex [Figure 4]. A lobulated mass with solid and cystic areas, calcification, and septations was seen in the right lobe of thyroid with hypermetabolic solid component (SUVma x 14.2) extending up to suprasternal region.
The imaging diagnosis was an aggressive right atrial angiosarcoma with neuroparenchymal, pulmonary, hepatic, pancreatic, renal and nodal metastases as the USG guided FNAC of thyroid swelling was suggestive of colloid goiter. After explaining the nature of the disease and poor prognosis, patient did not give consent for biopsy. Palliative chemotherapy with ifosfamide (7500 mg/m2, every 3 weeks) was instituted. Unfortunately, patient passed away after treatment with three cycles of chemotherapy.
18F-fluoro-deoxyglucose (FDG) contrast enhanced positron emission tomography-computed tomography (PET-CT) images. coronal CT and corresponding fused PET-CT sectional images showing non-FDG avid thrombus in right superior pulmonary vein extending upto the left atrium (arrow).