This case report demonstrates that while pediatric primary splenic angiosarcoma may be chemo-responsive to gemcitabine and docetaxel, it remains a highly aggressive and fatal disease without complete surgical extirpation.
Pediatric splenic angiosarcoma remains fatal without resection despite chemo-responsiveness; leaves open gemcitabine-docetaxel role in multimodal regimens.
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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Serrano et al. (2014) studied this question.
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