PMC5410477_01
The ECG findings. ECG shows sinus rhythm with a heart rate of 120 beats/min, low voltage, poor r wave progression in leads V1-3, a Q wave in lead V3, slight ST segment elevation in leads V2-5, and a negative T wave in leads V2-5 (a). A chest X-ray in the supine position shows right pleural effusion and pulmonary congestion (b). Left ventriculography (LVG) shows severe hypokinesis of the middle segment and apex and the preserved contraction of the basal segment of the left ventricle (c: end-diastolic phase, d: end-systolic phase).
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ECG showed sinus rhythm and a heart rate of 120 beats/min, low voltage, poor r wave progression in leads V1-3, a Q wave in lead V3, slight ST segment elevation in leads V2-5, and negative T waves in leads V2-5 (Fig. 1a). A chest X-ray in the supine position revealed right pleural effusion and pulmonary congestion (Fig. 1b). Left ventriculography (LVG) showed severe hypokinesis of the middle segment and apex, and the preserved contraction of the basal segment of the left ventricle (Fig. 1c, d).
Right ventriculography (RVG) shows severe hypokinesis (arrows), with the exception of the basal segment (arrow heads) of the right ventricle (a: end-diastolic phase, b: end-systolic phase).
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Right ventriculography (RVG) showed severe hypokinesis, with the exception of the basal segment of the right ventricle (Fig. 2).
The microscopic findings of a biopsy specimen from the endomyocardium of the right ventricle. a: Vacuolar degeneration (arrowheads) and a contraction band (thick arrows) are seen in the myocytes. Cellular infiltration is observed in the interstitium (thin arrows) (Hematoxylin and Eosin staining; scale bar, 50 mum). b: Azan staining also demonstrates cellular degeneration (violet-colored myocytes, arrows) and interstitial fibrosis (scale bar, 50 mum). c: Leukocyte common antigen (CD45) -positive cells are seen in the interstitium (scale bar, 50 mum). d: Polymorphonuclear cells (neutrophils) (arrowheads) and plasma cell (arrows) are also seen (scale 20 mum).
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The biopsied myocardium showed vacuolar degeneration and a contraction band in some myocytes (Fig. 3a). A small number of myocytes were necrotic, and fine fibrosis was also seen (Fig. 3b). Cellular infiltration was observed in the interstitium, and leukocyte infiltration was confirmed by the immunostaining of leukocyte common antigen (CD45) (Fig. 3c). Polymorphonuclear cells (neutrophils) and plasma cells were also seen (Fig. 3d).
Immunostaining of several types of infiltrating cells [a: LeuM1, b: CD3, c: L26 (CD20), d: CD68]. All types of cells are seen among the infiltrating cells, fewer B-cells are observed in comparison to other types of cells (scale bar, 50 mum).
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The proportions of the cell types was as follows: T-lymphocytes, 4; macrophages, 2; neutrophils, 2; B-lymphocytes, 1. (Fig. 4). Defects in the uptake of MIBG were also observed from the middle segment to the apex of the left ventricle, while the myocardial uptake of MIBG was maintained in the basal segment of the left ventricle (Fig. 4b, c).
CT, 123I-MIBG scintigraphy, and the histopathology of the adrenal tumor. A 30-mm right adrenal tumor (arrows) is observed on CT (a). The strong uptake of the adrenal tumor is observed on 123I-MIBG scintigraphy (b: anterior view, c: posterior view) (arrows). uptake of MIBG in the myocardium is low (arrowheads). The histopathological findings are consistent with pheochromocytoma (d: Hematoxylin and Eosin staining, scale bar: 50 mum, e: immunostaining with chromogranin A).
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CT showed a right adrenal tumor of 30 mm in diameter (Fig. 5). The strong uptake of 123I-metaiodobenzylguanidine (123I-MIBG) by the adrenal tumor was observed in 123I-MIBG scintigraphy on day 8 (Fig. 5b, c). A histological examination of the tumor with immunohistochemistry confirmed the diagnosis of pheochromocytoma (Fig. 5d, e).