Long-Term Survival due to Chemotherapy including Paclitaxel in a Patient with Metastatic Primary Splenic Angiosarcoma

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.

Primary splenic angiosarcoma: a diagnostic enigma

Primary splenic angiosarcomas (PSA) arise from splenic endothelium are rare and impose a diagnostic challenge preoperative. They can present as asymptomatic splenomegaly however; the commonest presentation is abdominal pain. The spleen can rapidly increase in size and can manifest as spontaneous rupture which would cause peritoneal dissemination of disease. Early metastasis of PSA is seen in liver, lungs, lymph nodes and gastrointestinal system. Preoperative diagnosis requires a high index of suspicion and ultrasound, contrast enhanced computerized tomography may essential to differentiate from splenic hemangioma. Splenic angiosarcoma are best treated with splenectomy with a limited disease, with care taken not to rupture and cause spillage. PSA are resistant to adjuvant radiation and chemotherapy. Mortality is high with median survival rate of only 5 months, irrespective of treatment and hence the need to diagnose before complications. Bisphosphonates, adjuvant radiation with chemotherapy have been attempted to increase disease free survival. We report a case of PSA emphasizes on early preoperative diagnosis to avoid progression of the disease.