Metastasized angiosarcoma of the spleen in a 2-year-old girl.

Primary angiosarcoma of the spleen is rare and the prognosis is very poor. The authors present a 2-year-old girl with spontaneous rupture of splenic angiosarcoma. At diagnosis there were liver metastases. After splenectomy she received chemotherapy with ifosfamide, vincristine, and actinomycine D combined with a partial liver resection. She remained in complete remission over 2 years from diagnosis.