Primary angiosarcoma of the spleen in a child with long-term survival

Primary angiosarcoma of the spleen is a very rare and aggressive neoplasm with poor prognosis. Splenic angiosarcoma rarely occurs in the pediatric group (18 years or younger). Survival duration for pediatric patients is very limited. We report a 7-year-old boy with splenic angiosarcoma presented with left upper quadrant abdominal pain and a palpable abdominal mass. Hematogram revealed anemia. Abdominal ultrasound revealed a heterogeneous mass about 12 cm in diameter in the left upper abdomen. Abdominal computed tomography demonstrated a huge heterogeneous mass with some cystic components closely attached to the spleen. Splenectomy was performed. Histological examination of the surgical specimen showed that this tumor had typical feature of angiosarcoma including vasoformative architecture, highly pleomorphic tumor cells with irregular, hyperchromatic and prominent nucleoli, and some mitosis. The postoperative course was uneventful. The patient was disease free at 16 years after surgery. This is the longest reported duration of survival in a patient with splenic angiosarcoma.