Summary

Primary splenic angiosarcoma, a very rare mesenchymal tumour of endothelial cell origin, comprises 2.6% of all cases of angiosarcoma and 10% of all primitive splenic tumours. Clinical presentation is usually unspecific, with abdominal pain and anaemia. Rupture is a rare complication and should prompt emergency splenectomy. Prognosis is usually poor because of liver, lung or bone metastases. We describe the case of an 80-year-old woman admitted to the emergency room with syncope, hypotension and vomiting. She stabilised after fluid resuscitation. Investigations showed anaemia, a large, heterogeneous spleen and free fluid in the abdominal cavity. She underwent emergency splenectomy. Pathology revealed primary splenic angiosarcoma. The postoperative period was complicated by respiratory failure but the patient made an otherwise uneventful course and was discharged 2 weeks after surgery. Six months after the operation she remains free of disease with no adjuvant treatment.