A primary splenic
angiosarcoma is a rare type of
soft tissue sarcoma and is associated with an extremely poor prognosis. In this study, we describe the case of a patient who was diagnosed with metastatic primary splenic
angiosarcoma and survived for about 2 years. A 62-year-old female was referred to us for the treatment of splenic
angiosarcoma with
disseminated intravascular coagulation (
DIC) and multiple liver and bone
metastases.
Paclitaxel therapy resulted in recovery from
DIC and enabled her to continue sequential treatment through to sixth-line
chemotherapy. We reviewed all splenic
angiosarcoma case reports which were described as stage IV to date and compared with our case. From these data, we found that the median overall survival was 105 days, and the prognosis of splenic
angiosarcoma of stage IV was worse than conventional case series.
Splenectomy was performed in more patients than
chemotherapy as a treatment. Moreover, various chemotherapeutic regimens were used. These data suggest that administering
chemotherapy including
paclitaxel to patients with splenic
angiosarcoma might improve their prognosis.