Cardiac angiosarcoma

Last revised by Bahman Rasuli on 30 Aug 2020

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

Please refer to the article on angiosarcomas for a general discussion about this entity.

They occur slightly more frequently in males. 

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

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

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

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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