Primary Angiosarcoma of the Spleen: Rare Diagnosis withAtypical Clinical Course

Primary angiosarcoma of the spleen is a rare diagnosis with poor prognosis. Morphologically, it demonstrates conventional bloodvessel differentiation. We present a case of 65-year-old female who underwent radical splenectomy for primary angiosarcoma ofthe spleen. After three-year disease-free interval, she was diagnosed with bone-only metastatic disease. Palliative radiotherapy andbisphosphonates kept her disease reasonably stable for another four years. After development of lung metastases, six cycles of singleagent doxorubicin kept her progression-free for six years. Upon further progression in lungs, thirteen years after original diagnosis,lung biopsy confirmed metastatic splenic angiosarcoma in the lungs. She started weekly paclitaxel chemotherapy. Although splenicangiosarcoma generally carries grave prognosis, some patients may enjoy prolonged periods of disease stabilization. Durable benefitcan be achieved in some patients with multimodality management. We review the literature focusing on systemic treatment for thisrare tumor.