A bleeding heart: case report and review of pericardial angiosarcoma

Abstract

Primary cardiac tumors are rare. The cardiac sarcomas are the most common malignant cardiac tumors. These tumors have a dismal prognosis with an overall median survival of 25 months. Clinical features include dyspnea, arrhythmias, pericardial effusions, heart failure, and sudden cardiac death. The diagnosis is often challenging. Therefore, the cardiac imaging workup plays a central role in addition to a high clinical suspicion in the setting of atypical presentations that do not respond to standard therapies. The echocardiography, computed tomography, and cardiac MRI are crucial in clinching the diagnosis. Multimodal treatment with surgery, chemotherapy, and radiotherapy has been shown to improve outcomes, as opposed to using either of these modalities alone. We describe the case of a 30-year-old gentleman with COVID-19 infection who developed recurrent hemorrhagic pericardial effusions refractory to standard treatment and was eventually diagnosed as a case of pericardial angiosarcoma after his biopsy revealed the diagnosis and staging was performed using PET-CT-FDG scan. Our case re-emphasizes the importance of considering a malignant etiology early in the course of the disease presentation, especially in recurrent hemorrhagic effusions despite an inflammatory cytologic diagnosis of fluid. It also highlights the place for cardiac CT and MRI to ascertain the location and spread and to plan the further course of treatment. If diagnosed early, the estimated survival time can be prolonged by instituting a multimodal approach.

Keywords: Cardiac Tamponade; Docetaxel; Gemcitabine; Pericardial Effusion; Sarcoma.

Figure 1. CT chest angiogram demonstrating moderate to large-sized bilateral pleural effusions, greater on the right side (green arrowheads), moderate-sized pericardial effusion (red arrowhead), thickened pericardium (yellow arrowhead), along with mediastinal and hilar adenopathy, and mediastinal fat stranding.

Figure 2. Photomicrograph of the pericardial biopsy. A – a low-power view of the malignant epithelioid neoplasm, highlighting the anastomosing pattern of vascular channels (H&E, 10X); B – highlights the neoplastic cells with hob-nailing, nuclear enlargement, pleomorphism, and hyperchromasia (H&E, 20X); C – highlights nuclear atypia and increase in mitotic activity of the tumor (H&E, 40X).

Figure 3. Photomicrograph of immunohistochemistry in low-power view (magnification 10X) with tumor cells showing strong and diffuse staining for ERG (A) and CD 31 (B).