PRIMARY ANGIOSARCOMA OF THE RIGHT ATRIUM AS A CAUSE OF CARDIAC TAMPONADE AND DEATH OF A 33-YEAR-OLD FEMALE PATIENT – AUTOPSY REPORT

Autori:

Tihana Džombeta, Marija Milković Periša, Tanja Leniček, Davor Tomas, Božo Krušlin

Sažetak
Iznenadna smrt najčešće nastaje kao posljedica oštećenja kardiovaskularnog sustava, ponajprije zbog koronarne bolesti ili kardiomiopatije, a izuzetno rijetko može biti uzrokovana primarnim tumorom srca. Tri četvrtine primarnih novotvorina srca čine benigni tumori, a četvrtinu maligni koji su redovito sarkomi i rijetko limfomi. Najčešći histološki tip primarnog sarkoma srca jest angiosarkom, obično smješten u desnom atriju ili rjeđe u perikardu. U ovom radu prikazujemo slučaj bolesnice koja je primljena u teškom općem stanju, pod sumnjom na septički šok, a preminula je nekoliko sati nakon prijma. Obdukcijom je nađen tumor desne pretklijetke koji je, prema morfološkim i imunohistokemijskim karakteristikama, odgovarao angiosarkomu. Infiltracija epikarda i krvarenje u perikardijalnu šupljinu doveli su do tamponade srca i smrti. Uz prikaz bolesnice rad pruža kratki pregled epidemioloških i kliničkih značajka te mogućnosti dijagnostike i liječenja primarnog angiosarkoma srca.
Summary

Sudden death most commonly occurs due to cardiac-related disorders, namely coronary artery disease or cardiomyopathies, while primary cardiac tumors are exceptionally rare. Three quarters of primary cardiac tumors are benign and the rest are malignant, mainly sarcomas and rarely lymphomas. The most common histological subtype of primary cardiac sarcoma is angiosarcoma, which is usually located in the right atrium and sometimes in the pericardium. Here, we report a case of a female patient admitted to our institution due to suspected septic shock, who died within a few hours of admission, despite given supportive measures.The autopsy revealed a tumor of the right atrium showing histological and immunohistochemical characteristics of angiosarcoma. Infiltration of epicardium and hemorrhage in the pericardial cavity caused cardiac tamponade and death. This report also provides a brief review of epidemiological and clinical features, as well as diagnostic and treatment options for primary cardiac angiosarcoma.