Primary splenic
angiosarcoma is a very rare
neoplasm with a high propensity for metastatic disease and poor prognosis. There is a paucity of literature concerning this specific
sarcoma subtype and the role of systemic
therapy is not well defined. A retrospective review of the prospectively maintained University of Washington/Seattle
Cancer Care Alliance
Sarcoma Unit database was performed to identify patients with splenic
angiosarcoma treated between 2007 and 2012. In total there were 19 patients with
angiosarcoma treated at the Seattle
Cancer Care Alliance from 2007 to 2012. The number of patients with splenic
angiosarcoma was 2 (11%). The first patient was a woman aged 57 years who was referred with metastatic splenic
angiosarcoma to the liver, post-
splenectomy. She was treated with 4 cycles of weekly
paclitaxel prior to metastatic resection and 4 cycles of the same
drug in an adjuvant scenario, achieving a pathological complete response to treatment. She is alive and on third-line systemic
therapy. The second patient was a male patient aged 30 years who presented with metastatic high-grade splenic
angiosarcoma and was treated with 3 lines of systemic
therapy, including
doxorubicin,
paclitaxel and gemcitabine+docetaxel, but developed a gastrointestinal
metastasis with subsequent gastrointestinal
bleeding. Splenic
angiosarcoma is a very rare
neoplasm. Surgery remains the mainstay of management for localized disease.
Paclitaxel administered weekly proved to be well-tolerated and resulted in a good radiological response in one of our patients, enabling resection of metastatic disease. Durable clinical benefit can be achieved in metastatic splenic
angiosarcoma with multi modality management.