Primary angiosarcoma of the pericardium is exceptionally rare, and diagnosis remains challenging because of the variability of its clinical presentation. Here, we report a case of a 26-year-old woman with pericardial angiosarcoma who presented with recurrent hemorrhagic pericardial effusion diagnosed at histopathologic examination.

A 26-year-old female patient had a 4-month history of dyspnea, chest pain, cough, palpitation, and fatigue. Echocardiography revealed pericardial effusion. However, no obvious mass was identified either in the pericardium or in the heart. Pericardiocentesis and drainage were performed 3×, draining 700 mL of hemorrhagic pericardial effusion. The patient’s symptoms improved after percutaneous drainage of bloody pericardial fluid. The effusion cultures for bacteria, acid-fast bacilli, and viruses were always negative. Repeated cytological examination of the pericardial effusion did not show malignant cells. Her dyspnea and cough recurred after 2 months. Echocardiography showed thickened pericardium and mild pericardial effusion. The patient was empirically treated for tuberculous pericarditis, considering the unexplained hemorrhagic pericardial effusion and the high incidence of tuberculous pericarditis in China. After 1 month, she did not respond to antituberculous therapy, and her clinical condition rapidly deteriorated. Therefore, she was referred to our institution for further examination.

On physical examination, her pulse rate, respiration rate, and blood pressure were 127 beats per minute, 22 per minute, and 117/65 mm Hg, respectively. Kussmaul sign and paradoxical pulse were present. No cardiac murmur or pericardial rub was detected.

Chest radiograph showed marked cardiomegaly (Figure [A]). Transthoracic echocardiography revealed a huge mass in the pericardium surrounding and compressing the ventricles, atria, ascending aorta, and pulmonary trunk (Figure [B] and [C]; Movies I through III in the Data Supplement). Her echocardiography demonstrated septal bounce and dilation and diminished collapse of the inferior vena cava. Color Doppler flow imaging showed mild tricuspid regurgitation (Figure [D]; Movie IV in the Data Supplement). Pulsed-wave Doppler echocardiography demonstrated that peak mitral E inflow decreased (>25%) after inspiration (Figure [E]). High enhancement of contrast agent in the pericardial mass was noted after contrast echocardiography (Figure [F]; Movie V in the Data Supplement). Chest computed tomography showed that the entire heart was encased by the pericardial mass. Cardiac magnetic resonance imaging revealed that the large mass within the pericardium surrounded and severely compressed the entire heart and great vessels. On T1- and T2-weighted cardiac magnetic resonance images, the pericardial mass appeared heterogeneous and hyperintense relative to the myocardium (Figure [G], [H], and [I]). After intravenous contrast with gadopentetate dimeglumine, enhancement was observed as multiple lines in the tumor radiating from the epicardium to pericardium (a sunray appearance; Figure [J]). Positron emission tomography revealed a neoplastic mass in the pericardium (Figure [K]). Distant metastases were found only in the lymph node of the right cardiophrenic angle and the left musculus obliquus externus abdominis (Figure [L]).

Figure. Multimodal imaging and pathological findings.A, Chest radiograph showing marked cardiomegaly. B and C, Transthoracic echocardiography showing a large pericardial mass surrounding and compressing ventricles and atria. D, Color Doppler flow imaging indicates mild tricuspid regurgitation. E, Pulsed-wave Doppler echocardiography demonstrates that peak mitral E inflow decreased (>25%) after inspiration. F, Contrast echocardiography showing high enhancement of contrast agent in the pericardial mass. G, The pericardial mass appears heterogeneous and hyperintense relative to the myocardium on T1-weighted cardiac magnetic resonance (CMR) images. H and I, The pericardial mass is heterogeneous and hyperintense on T2-weighted CMR images. J, Enhanced CMR imaging reveals multiple lines in pericardial tumor, radiating from the epicardium to pericardium (sunray appearance). K and L, Positron emission tomography reveals a neoplastic mass in the pericardium and distant metastases. Ao indicates aorta; Ee, E velocity during expiration; Ei, E velocity during inspiration; LA, left atrium; LV, left ventricle; M, mass; RA, right atrium; and RV, right ventricle.

Open biopsy was performed for a definitive diagnosis. Histopathologic examination showed a malignant tumor. Immunohistochemical staining indicated that these cells were positive for CD (cluster of differentiation) 31, CD34, ETS-related gene, and Ki67. Hence, the overall findings were consistent with pericardial angiosarcoma. The patient refused chemotherapy and radiotherapy, considering the distant metastases of pericardial angiosarcoma and poor prognosis, and she died after 2 months.

Primary cardiac tumors are rare entities (0.0017%–0.003% of routine autopsy studies, 25% malignant).¹ Cardiac angiosarcomas, although rare, are the most common primary malignant cardiac tumors (31% of all malignancies).² They tend to be found in patients aged 20 to 50 years and are more common in men.³ Most cases are more often found in the right atrium, making pericardial angiosarcoma extremely rare. Pericardial angiosarcomas respond poorly to chemotherapy. Prognosis is poor, with mean survival time of 6 to 11 months.⁴

Making an accurate diagnosis remains challenging because its clinical presentation is variable, and available noninvasive imaging modalities lack specificity. Echocardiography, although frequently performed, plays a limited role in the evaluation of primary pericardial angiosarcoma. Echocardiography frequently demonstrates pericardial effusion but may not show a mass because it depends on good acoustic window. Pericardiocentesis commonly yields hemorrhagic fluid that does not contain malignant cells, as seen in our case. Computed tomography demonstrates the location, size and extent of the tumor, and its invasion of vital structures. Cardiac magnetic resonance imaging is a more powerful tool for characterizing the lesion. Cardiac magnetic resonance imaging may also demonstrate constrictive physiology with diffuse thickening of the pericardium. Positron emission tomography may be a useful tool because it may demonstrate distant or locoregional metastatic disease. Detecting this malignant tumor in the early stage may be challenging, despite the application of various advanced imaging modalities.

We describe a case of a patient with pericardial angiosarcoma who presented with recurrent hemorrhagic pericardial effusion that rapidly progressed in a short period. Our case indicates that unexplained hemorrhagic pericardial effusion should prompt the clinician to seek for a malignant etiology, even when malignant cells are not found on fluid cytology.

This work was supported by the National Natural Science Foundation of China (No. 81401432 and 81727805).

None.

https://www.ahajournals.org/journal/circheartfailure

The Data Supplement is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCHEARTFAILURE.118.005342.