Angiosarcoma is a rare mesenchymal neoplasia with an incidence of only 0.14-0.25 cases per million [1]. Most common sites of occurrence are skin and superficial soft tissues, followed by breast, liver, spleen, and bone. Early stage metastases to lung, liver, and lymph nodes are common and overall prognosis is poor [2, 3]. Only approx. 5% of angiosarcomas originate from the spleen, and the existing casuistic reports suggest a dismal clinical prognosis. Here, we describe a rare case of splenic angiosarcoma leading to thrombocytopenia as first manifestation of the disease.
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