PMC7843435_01
(A) Computed tomography pulmonary angiography showed multiple embolisms of bilateral pulmonary arteries (arrow) with a mild-sized pericardial effusion. (B) Computed tomography showed a severe-sized pericardial effusion and bilateral pleural effusion after heparin anticoagulation. (C) The left pleural effusion was increased after pericardial drainage. (D) Computed tomography revealed mild bilateral pleural effusion and pericardial effusion after drainage. (E) Contrast-enhanced computer tomography showed a mixed mass located in the right pericardium (4.5 x 8.5 cm), and the boundary was unclear between the mass and the right atrial wall, heterogeneous thickened pericardium, and moderate pericardial effusion locally. (F) Multiple metastatic nodules surrounded by a wide range of ground-glass-like effusion (diffuse alveolar hemorrhage) were identified in the bilateral lungs. M, mass; RA, right atrium.
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F1
Computed tomography pulmonary angiography (CTPA) showed multiple embolisms of bilateral pulmonary arteries and a mild-sized pericardial effusion (Figure 1A). Both transthoracic echocardiogram (TTE) and thoracic CT were significant for severe large-scale pericardial effusion (Figure 1B). Repeated thoracic CT the following day was significant for bilateral pleural effusion, which was greatly increased in amount (Figure 1C). Repeated thoracic CT prior to discharge showed mild bilateral pleural effusion and pericardial effusion (documented on December 30, 2019; Figure 1D). However, CTPA also showed a large mixed mass, occupying the right pericardium (4.5 x 8.5 cm; Figure 1E), and she was readmitted. CCTA also showed a mild left pleural effusion, enlarged mediastinal and hilar lymph nodes, multiple and scattered ground-glass opacity [diffuse alveolar hemorrhage (DAH)] nodules over bilateral lung field, and multiple bipleural nodules (Figure 1F, May 21, 2020).
Transthoracic echocardiogram revealed a huge mass in the pericardium surrounding and compressing the right atrium and right ventricle (A,B). Mild blood flow signals (arrow) were seen in the mass (A) (May 20, 2020). The tumor was significantly larger than before and strip-like hyperechoism (arrow) inside the right atrium (B) (June 18, 2020). Contrast-enhanced ultrasound revealed the mass transferred right atrium, and the mass was rapidly filled with contrast agent (C1-C3) (May 20, 2020). Axial Fiesta (D) and 4CH-2D (E) images of cardiac magnetic resonance showed an irregular, large tumor (size up to 10 x 5.5 cm) with heterogeneous distinction diffusely within the pericardium (10 x 5.5 cm), mainly locating outside of right cardiac system, and compressed the right heart. RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle; M, mass; RA, right atrium.
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F2
Mild blood flow signals were also observed in the mass (Figure 2A) (May 20, 2020). The tumor was significantly larger than before with a strip-like hyperechoism within the RA (Figure 2B, recorded on June 18, 2020). Contrast-enhanced ultrasound showed the mass and chamber were filled with contrast agent almost simultaneously (Figures 2C1-C3 and Supplementary Movie 2). Axial Fiesta (Figure 2D) and 4CH-2D (Figure 2E) of cardiac magnetic resonance (CMR) showed an irregular, large tumor (size up to 10 x 5.5 cm) with heterogeneous distinction diffusely within the pericardium (10 x 5.5 cm), mainly located outside the right cardiac chamber system, compressing the right heart.
Positron emission tomography scan showed a heterogeneous mass at the right margin of pericardium with circular hypermetabolic uptake of fluorodeoxyglucose-18, poorly demarcated from the right atrium (A-C). Distant metastases were found in both lungs, bilateral pleural (A,D), nodules in hilar and mediastinal lymph (E,F) (arrow).
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Contrast-enhanced ultrasound showed the mass and chamber were filled with contrast agent almost simultaneously (Figures 2C1-C3 and Supplementary Movie 2). Positron emission tomography scan exhibited a heterogeneous mass located at the right margin of pericardium, poorly demarcated from the RA and superior vena cava (Figures 3A-C). Distant metastases were found in both lungs and bilateral pleura (Figures 3B,D), nodules in hilar and mediastinal lymph nodes (Figures 3E,F).
Biopsy of pericardial mass showed pleomorphic spindle and ovoid tumor cells with atypia and mitotic [hematoxylin-eosin stain, original magnification x400, (A)]. The malignant cells were strongly positive for CD31 [blue, x200, (B)] and CD34 [blue, x200, (C)] in immunohistochemistry.
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F4
The biopsy specimen showed a malignant tumor in histopathologic examination (Figure 4A) and positive immunohistochemical staining for CD31 (Figure 4B) and CD34 (Figure 4C), but negative for CEA, CK, CK5/6, CR, D2-40, desmin, and MC, indicating poorly differentiated angiosarcoma.
Progression and examination of the clinical course. PTE, pulmonary thromboembolism; BNP, brain natriuretic peptide; CTPA, computed tomography pulmonary angiography; TTE, transthoracic echocardiogram; CMR, cardiac magnetic resonance; PET, positron emission tomography; CCTA, coronary computed tomography angiography.
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F5
The progression and examination of the clinical course are shown in Figure 5.