Vague Respiratory Symptoms as the Initial Presentation of Primary Cardiac Angiosarcoma: A Case Report

Abstract

Primary cardiac angiosarcoma is a rare and aggressive tumor. Its symptoms and signs are often diverse and nonspecific, often leading to delayed diagnosis. Here we report a case of primary cardiac angiosarcoma with vague respiratory symptoms as the first presentation, possibly due to pulmonary metastasis.

The patient is a 58-year-old woman with shortness of breath for several weeks.  She further developed a dry nonproductive cough. Other symptoms include diarrhea, nausea and vomiting, fever, and fatigue.  Her past medical history included ankylosing spondylitis, fibromyalgia, hypertension, and tobacco use. Chest CT without contrast showed numerous bilateral pulmonary nodules scattered throughout her lungs. The main clinical differential diagnosis includes infectious endocarditis with septic pulmonary emboli or other possible multifocal infections. However, a computed tomography angiography (CTA) showed no evidence of pulmonary embolic disease. After being admitted into the hospital, a 2-dimensiomal echocardiogram was performed, revealing a sessile ovoid mass in the right atrium measuring 3.5 x 4.3 cm. MRI confirmed the above findings.

A CT-guided left lower lobe lung biopsy was performed, revealed lung parenchyma with areas of vascular proliferation lined by atypical cells showing high nuclear/cytoplasmic ratio, hyperchromatic nuclei, and frequent mitosis. These atypical cells are positive for CD31 and CD34, consistent with endothelial origin. TTF-1, CK7, CAM5.2, and CAMTA1 were all negative. Subsequently, a right atrial biopsy was also performed, showing scant neoplastic cells with significant nuclear atypia, frequent mitotic figures, and frequent intracytoplasmic vacuoles. The neoplasm appeared to be forming vaguely vascular spaces in a few minute areas combined with a diffuse pattern of growth, consistent with a neoplasm of vascular origin. Similar to the lung biopsy, neoplastic cells from right atrium are strongly positive for CD31, focal positivity for CD34, and negative for TTF-1, BER-EP4, MOC-31, CK5/6, calretinin, and HHV8. In addition, MIB-1 expression showed a high proliferative index. CAMTA immunostaining for epithelioid hemangioendothelioma was also negative. Based on the overall clinical, radiological, histological and immunohistochemical features, a final diagnosis of cardiac angiosarcoma with multifoci of lung metastasis was made. Outside pathological consultations agreed with this diagnosis.

Because the clinical presentation of cardiac angiosarcoma can be variable and nonspecific, raising awareness that the vague respiratory symptoms as one of its first presentation can avoid delayed diagnosis.

[N A J Med Sci. 2024;17(1):019-024.   DOI: 10.7156/najms.2024.1701019]

Key Words: Cardiac angiosarcoma, vascular neoplasm, lung metastasis, malignant tumor