Amplification Defines a Genetically Distinct Subset of Angiosarcomas.

Angiosarcoma is a rare, devastating malignancy with few curative options for disseminated disease. We analyzed a recently published genomic data set of 48 angiosarcomas and noticed recurrent amplifications of -cluster genes in 33% of patients. genes are master regulators of embryonic vascular development and adult neovascularization, which provides a molecular rationale to suspect that amplified genes act as oncogenes in angiosarcoma. amplifications typically affected multiple pro-angiogenic genes and co-occurred with amplifications of and whereas the overall mutation rate in these tumors was relatively low. amplifications were found most commonly in angiosarcomas located in the breast and were rare in angiosarcomas arising in sun-exposed areas on the head, neck, face and scalp. Our data suggest that -amplified angiosarcoma is a distinct molecular subgroup. Efforts to develop therapies targeting oncogenic gene expression in AML and other sarcomas may have relevance for -amplified angiosarcoma.