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Case Reports
. 2019 Jul 30:2019:2641976.
doi: 10.1155/2019/2641976. eCollection 2019.

Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review

Dan Morgenstern-Kaplan  1 , Carlos Manuel Aboitiz-Rivera  2 , Ruben Blachman-Braun  1 , María Eugenia Vázquez-Manríquez  3 , Benito Sarabia-Ortega  2 , Mario Enrique Baltazares-Lipp  2
Affiliations
Case Reports

Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review

Dan Morgenstern-Kaplan et al. Case Rep Oncol Med. .

Abstract

Cardiac angiosarcoma is a rare and clinically challenging pathology. It is a high-grade primary malignant tumor of the heart tissue that has many variants, of which the epithelioid variant is rarely present in the heart or the great vessels. As with many other cardiac tumors, it is mainly a diagnosis of exclusion and the initial diagnostic test is an echocardiogram followed by a biopsy with immunohistochemistry analysis to ascertain the type of tumor. The differential diagnosis of cardiac tumors is challenging due to the overlapping clinical manifestations with different cardiac tumors and systemic diseases. Cardiac angiosarcomas are often aggressive with a poor prognosis even with treatment. Herein, we present a case of the epithelioid variant of a cardiac angiosarcoma in addition to a thorough review of the recent literature on the clinical manifestation, diagnosis, and treatment of this type of tumors.

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Conflict of interest statement

The authors declare that there are no conflicts of interest regarding the publication of this article.

Figures

Figure 1
Figure 1
Chest CT scan with IV contrast showing a hyperdense mass within the pericardial space, with infiltration into the myocardium, displacing and compressing adjacent structures.
Figure 2
Figure 2
Transthoracic echocardiography. (a) Parasternal short axis view showing tumoral infiltration around the aortic root without obstruction of the outflow tract. (b) Apical 4-chamber view showing myocardial infiltration to the interatrial septum and the free wall of the right atrium and ventricle.
Figure 3
Figure 3
(a) Microphotography H&E (20x). In the center, we can observe a blood vessel surrounded by neoplastic cells in two different patterns: cells forming nests and spindle cells fusing with each other. (b) Microphotography H&E (40x). The tumoral cells are shown in detail, with a medium to large size, scant cytoplasm, oval nuclei of vesicular chromatin, prominent nucleolus, and several mitotic figures. (c) Immunochemistry of the CD31 marker: the neoplastic cells' cytoplasm and membrane are positive, while the bronchial epithelium is negative.
See this image and copyright information in PMC

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