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11 May 2011 Case of the Week #206
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Case of the Week #206
An 80 year old man was admitted for anemia and angina. Workup revealed hepatosplenomegaly and multiple splenic infarctions. He got worse, and a splenectomy was performed. Further studies showed multiple lesions in bones, the gastrointestinal tract, pancreas, liver, peritoneum, lungs and both kidneys. Two days after the surgery, the patient died. An autopsy was performed.
The left two images are from the abdominal aorta
What is your diagnosis?
Intimal sarcoma of abdominal aorta with multiple tumor emboli and metastasis with angiosarcomatous differentiation
Intimal sarcoma is defined as a malignant tumor arising in the tunica intima of large blood vessels. Most cases develop in the aorta, and are undifferentiated. In undifferentiated cases, there is no gender preference, and mean age is 66 years (Am J Surg Pathol 2005;29:1184). Tumors are typically largely necrotic, with poorly differentiated epithelioid and pleomorphic cells associated with the tunica intima. Tumors are considered to be of endothelial origin, due to immunoreactivity for CD31 (Am J Surg Pathol 1988;12:798) and FLI1, with variable staining of other markers.
Metastatic tumors tend to have different morphology from the primary tumor, including angiosarcomatous (as in this case), osteosarcomatous or rhabdomyosarcomatous differentation.
Most patients have metastatic disease and die within 1 year of diagnosis. Tumors typically present with thromboemboli (J Bras Pneumol 2009;35:814), and post-mortem diagnosis is common (Hum Pathol 1997;28:1306)
Nat Pernick, M.D., President
and Liz Parker, B.A., Associate Medical Editor
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