NCI Definition: A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma. [1]

Angiosarcomas most frequently harbor alterations in MYC, TP53, KDR, FLT4, and CRKL [2].

Most Commonly Altered Genes in Angiosarcoma

MYC Amplification, TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, and KDR Mutation are the most common alterations in angiosarcoma [2].

Top Alterations in Angiosarcoma

Significant Genes in Angiosarcoma

CD274 +



Disease Details

HEMANGIOSARCOMA, MALIGNANT, Malignant Hemangioendothelioma, Malignant Angioendothelioma, Hemangiosarcoma
Thyroid Gland Angiosarcoma, Pulmonary Artery Angiosarcoma, Prostate Angiosarcoma, Splenic Angiosarcoma, Breast Angiosarcoma, Ovarian Angiosarcoma, Skin Angiosarcoma, Aortic Angiosarcoma, Conventional Angiosarcoma, Bone Angiosarcoma, Gallbladder Angiosarcoma, Central Nervous System Angiosarcoma, Liver Angiosarcoma, Superior Vena Cava Angiosarcoma, Mediastinal Angiosarcoma, and Childhood Angiosarcoma
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1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.