PMC5914282_01
Specific radiographic imaging of the patient. (A,B) Trans-esophageal echocardiography showed functional single ventricle and severe common atrioventricular valve regurgitation. (A) CAVVs were open in cardiac diastole, and huge ventricular septal defect (white star) was observed. (B) CAVVs were closed in cardiac systole and regurgitation of blood into the ventricle (black star) was observed. RV, right ventricle; LV, left ventricle; CAVV, common atrioventricular valve. (C) Contrast-enhanced computed tomography sagittal reconstruction image of the abdomen showed a rounded mass (1.1 cm x 1.1 cm x 1.1 cm) in right adrenal gland (red arrow) and another mass (4.4 cm x 3.2 cm x 4.3 cm) with mixed density (black arrow) anterior to inferior vena cava (green arrow) at L2-3 lumbar level. The right kidney was showed with blue arrow. (D)
123I-MIBG scintigraphy showed intense uptake in the right adrenal gland (red arrow) and in front of the inferior vena cava (black arrow) at L2-3 lumbar level.
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F1
Recent echocardiography (Figures 1A,B) showed double outlet right ventricle, complete atrioventricular canal defect, severe common atrioventricular valve regurgitation, and pulmonary valve stenosis, indicating a functional single atrium and ventricle. A computed tomography (CT) scan of the abdomen and pelvis (Figure 1C) demonstrated a rounded mass (1.1 cm x 1.1 cm x 1.1 cm) in the right adrenal gland and another mass (4.4 cm x 3.2 cm x 4.3 cm) with mixed density anterior to inferior vena cava at L2-3 lumbar level. Marked accumulation of 123I-metaiodobenzylguanidine (MIBG) (Figure 1D) in the right adrenal gland and in front of the inferior vena cava was found.
Gross appearance and histopathology of the resected pheochromocytoma and paraganglioma masses. (A,B) The right adrenal gland nodule was about 1.2 cm in diameter, connecting to part of normal adrenal gland tissue. Hematoxylin and eosin staining (C), chromogranin (E), S-100 (G), and HIF-2alpha (I) in the right adrenal gland nodule. (A,B) The retroperitoneal mass was about 4.6 cm x 3.3 cm x 4.5 cm in size and the section showed partial necrosis. Hematoxylin and eosin staining (D), chromogranin (F), S-100 (H), and HIF2alpha (J) in the retroperitoneal mass.
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F2
The resected right adrenal gland nodule was 1.2 cm in diameter (Figures 2A,B). The retroperitoneal mass was 4.6 cm x 3.3 cm x 4.5 cm (Figures 2A,B) with multifocal areas of necrosis on the cut surface. Microscopically, the tumor cells (Figures 2C,D) had slightly basophilic cytoplasm. The immunohistochemistry test was positive for chromogranin (Figures 2E,F), S-100 (Figures 2G,H), and HIF2alpha (Figures 2I,J).