Revision 9 for 'Cardiac angiosarcoma'

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Cardiac angiosarcoma

Cardiac angiosarcomas are the most common sarcoma involving the heart (see cardiac tumors). 

Please refer to the article of angiosarcomas, for a general discussion.

Epidemiology

They occur slightly more frequently in males. 

Clinical presentation

Patients usually present with right-sided heart failure or cardiac tamponade

Pathology

These tumors tend to occur in the right atrium and involve the pericardium.

Radiographic features

Two main morphologic types have been described in angiosarcoma:

  1. well-defined mass protruding into a cardiac chamber, usually the right atrium
    • CT shows a low-attenuation right atrial mass, which may be irregular or nodular (usually arises from the right atrial free wall)
    • contrast material enhancement is heterogeneous
  2. diffusely infiltrative mass extending along the pericardium
    • pericardial space may be obliterated with hemorrhagic, necrotic tumor debris

Treatment and prognosis

Prognosis is poor, which may be due in part to the delay in diagnosis (patients usually have metastatic involvement at presentation).

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