Spontaneous Rupture of Primary Angiosarcoma of the Spleen

Primary angiosarcoma. of the spleen is an extremely rare malignancy, the pathogenesis of which is not completely understood, with high metastatic potential and an exceedingly poor prognosis, regardless of treatment regimen. The major complication is splenic rupture, which often leads to fatal hemoperitoneum. Overall, since 1879 when Langerhans described the first case of angiosarcoma of the spleen, there have been approximately 200 cases reported in the literature. Moreover, to the best of our knowledge, spontaneous rupture of primary splenic angiosarcoma and spontaneous rupture of remnant or recurred angiosarcoma is extremely rare, and no cases were reported in English literature. We report a case of spontaneous splenic rupture due to angiosarcoma in a 68-year-old man, and also review the existing literature. (J Korean Surg Soc 2009;77:291-295)