Primary hemangiosarcoma of the spleen: report of four cases

Four cases of primary splenic hemangiosarcoma are described. The most common presenting symptoms were recurrent, fever, malaise, and abdominal pain. Three patients also had complaints referable to the urinary tract. Severe hemolytic anemia with bizarre or fragmented red blood cells on peripheral smear was noted in three patients, in two cases, for more than 1 year before diagnosis. Two patients had been treated for mammary carcinoma with ionizing radiation 10 years before developing hemangiosarcoma of the spleen. One patient had multiple congenital hemangiomas present from birth, and is thought to have developed hemangiosarcoma in a previously existing benign hemangioma. None of our patients survived more than 7 months after the diagnosis of hemangiosarcoma. The importance of early detection and treatment of this tumor is discussed.