Angiosarcoma of the spleen presenting as spontaneous splenic rupture

INTRODUCTION: Angiosarcoma of the spleen is a rare malignancy of vascular origin with a high rate of metastasis and poor prognosis. We report one such rare case of spontaneous splenic rupture, along with a review of current literature. PRESENTATION OF CASE: A 30 year old man presented to our emergency services with severe abdominal pain, distension, hypotension and splenomegaly. Investigations revealed a marked anaemia, coagulopathy, severe lactic acidosis, and acute kidney injury. Imaging demonstrated splenomegaly with acute haemorrhage and lymphadenopathy. Laparotomy and splenectomy revealed piecemeal spleen and nodular omentum. The patient suffered an intra-operative cardiorespiratory arrest, and despite successful resuscitation, fatally arrested postoperatively in ICU. Histology revealed a primary splenic angiosarcoma with omental metastases. DISCUSSION: Primary splenic angiosarcoma was first reported in 1879, with only 200 cases reported to date, largely as isolated case reports, with an annual incidence of 0.14-0.25 per million. With variable symptomatology and a potential to present with life-threatening complications, early diagnosis is paramount. CT scanning shows distinctive changes and is invaluable in disease assessment. Tissue diagnosis is often possible only after splenectomy. Spontaneous rupture carries the worst prognosis. CONCLUSION: Primary splenic angiosarcoma is a rare and aggressive malignancy that often presents with metastatic disease, and largely carries a dismal prognosis. Definitive diagnosis is challenging, but imaging with CT scanning can show characteristic changes and establish any metastatic disease. With no established adjuvant therapy long term outlook remains poor even if treated successfully by surgery.