• Malignant tumor arising in tunica intima of large blood vessels, usually aorta
• Usually undifferentiated, mean age 66 years, no gender preference (Am J Surg Pathol 2005;29:1184)
  
• Tumors typically present with thromboemboli (J Bras Pneumol 2009;35:814), and post-mortem diagnosis is common (Hum Pathol 1997;28:1306)
• Most patients have metastatic disease and die within 1 year of diagnosis

• 69 year old man with rupture of descending thoracic aorta due to the necrosis of aortic intimal sarcoma (Interact Cardiovasc Thorac Surg 2010;10:462)
• 80 year old man with multiple splenic infarcts (Case of the Week #206)

80 year old man with multiple splenic infarcts Left two images are from abdominal aorta

• Tumors are typically largely necrotic, with poorly differentiated epithelioid and pleomorphic cells associated with tunica intima
• Metastatic tumors tend to have different morphology from primary, including angiosarcomatous, osteosarcomatous or rhabdomyosarcomatous differentation

80 year old man with multiple splenic infarcts

Causing aortic rupture

• CD31+ (Am J Surg Pathol 1988;12:798), FLI1

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