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The patient had a favorable tumor response to pembrolizumab. The patient's tumor burden remains stable at 5years following splenectomy. Angiosarcoma of the spleen is a rare clinical entity and is often challenging to diagnose early. Moratality is high, especially in the case of metastasis or spontaneous rupture. Due to the rare nature of this tumor, optimal treatment is not known. Here, we show excellent response in two patients to surgery combined with adjuvant therapy. Due to the rare nature of this tumor, optimal trea