What is the pathophysiology of an angiosarcoma (AS)?

Updated: Dec 02, 2020
  • Author: Maria Belén Carsi, MD, PhD, FRCS; Chief Editor: Edwin Choy, MD, PhD  more...
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Answer

Angiosarcomas arising at different sites and in different organs have some distinct features. Angiosarcomas may occur in any region of the body but are more frequent in skin and soft tissue. Angiosarcomas also can originate in the liver, breast, spleen, bone, or heart. [1, 2, 3, 4]

The Angiosarcoma Project performed whole-exome sequencing of 47 tumors and found recurrent mutations of genes including KDRTP53, and PIK3CA. PIK3CA-activating mutations were observed predominantly in primary breast angiosarcoma, while angiosarcoma of the head, neck, face, and scalp was associated with a high tumor mutation burden and a dominant ultraviolet damage mutational signature, suggesting that ultraviolet damage may be a causative factor and that immune checkpoint inhibition may be beneficial. [5]


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