Primary splenic angiosarcoma is an extremely rare and aggressive neoplasm of the vasculature. Uniformly, primary splenic angiosarcoma is a fatal disease despite early diagnosis and treatment. Only patients with localized disease amenable to surgical resection achieve long-term, disease-free survival. We present a review of the literature and report a case of a 3-year-old girl with metastatic primary splenic angiosarcoma who was offered aggressive surgical and medical treatment with curative intent despite her advanced presentation.
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