Metastatic renal angiosarcoma to the liver with underlying JAK2 mutation

Renal angiosarcomas are rare malignancies with approximately 70 published cases and comprise less than 1 % of all kidney tumors. We present an elderly male who was found to have a large 10 cm kidney mass and underwent a left radical nephrectomy. Pathology revealed a renal angiosarcoma and unfortunately 3 months later was found to have metastatic disease in the liver. He was incidentally found to have a JAK2 positive myeloproliferative neoplasm at the same time. Molecular testing on the angiosarcoma revealed a JAK2V617F mutation. He was treated with standard of care chemotherapy; initially with paclitaxel and then anthracyclines. He also received liverdirected therapy with Y90 with stable disease. He unfortunately passed away due to an unrelated illness. Our case highlights a rare type of kidney malignancy and that JAK2V617F is a potential driver mutation in angiosarcomas.