Overview

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 TP53, MYC, KDR, PIK3CA, and FLT4 [2].

Most Commonly Altered Genes in Angiosarcoma

TP53 Mutation, MYC Amplification, TP53 c.217-c.1178 Missense, TP53 c.142-c.212 Missense, and TP53 c.1-c.137 Missense are the most common alterations in angiosarcoma [2].

Top Alterations in Angiosarcoma

Significant Genes in Angiosarcoma

PIK3CA +

PTEN +

Disease Details

Synonyms
Hemangiosarcoma, HEMANGIOSARCOMA, MALIGNANT, Malignant Angioendothelioma, Malignant Hemangioendothelioma
Parent(s)
Malignant Vascular Neoplasm
Children
Superior Vena Cava Angiosarcoma, Bone Angiosarcoma, Gallbladder Angiosarcoma, Childhood Angiosarcoma, Ovarian Angiosarcoma, Splenic Angiosarcoma, Central Nervous System Angiosarcoma, Breast Angiosarcoma, Mediastinal Angiosarcoma, Conventional Angiosarcoma, Liver Angiosarcoma, Skin Angiosarcoma, Prostate Angiosarcoma, Thyroid Gland Angiosarcoma, and Aortic Angiosarcoma
OncoTree Name
Angiosarcoma
OncoTree Code
ANGS

References

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 4. 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.