| Summary: | 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 <i>HOXA</i>-cluster genes in 33% of patients. <i>HOXA</i> genes are master regulators of embryonic vascular development and adult neovascularization, which provides a molecular rationale to suspect that amplified <i>HOXA</i> genes act as oncogenes in angiosarcoma. <i>HOXA</i> amplifications typically affected multiple pro-angiogenic <i>HOXA</i> genes and co-occurred with amplifications of <i>CD36</i> and <i>KDR,</i> whereas the overall mutation rate in these tumors was relatively low. <i>HOXA</i> 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 <i>HOXA</i>-amplified angiosarcoma is a distinct molecular subgroup. Efforts to develop therapies targeting oncogenic <i>HOX</i> gene expression in AML and other sarcomas may have relevance for <i>HOXA</i>-amplified angiosarcoma.
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