Primary splenic angiosarcoma: a diagnostic enigma

ABSTRACT

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.