Primary Angiosarcoma of the Spleen

Abstract

The CT and MR findings in 2 patients with surgically proven splenic angiosarcoma are presented. Two distinctive radiologic and pathologic patterns were observed: total tumor replacement of the spleen with preservation of normal contour or multiple nodular masses in the spleen. The various findings on CT and MR imaging reflected the hemorrhagic nature of the lesions with increased iron content. MR imaging appeared to be superior to CT in demonstrating these features. In the case of focal lesions, radiologic differentiation from lymphoma or metastasis was impossible.

Angiosarcoma: unusual cause of acute abdominal pain and hemoperitoneum

“…Angiosarcoma predominantly affects the skin and soft tissues (57 %) and less frequently liver, breast, bone marrow, and spleen (4 % of the total) [3,4]. Primary splenic angiosarcoma, a rare condition of unknown pathogenesis arising from the endothelial lining of blood vessels, was first described by Langhans in 1879, and since then approximately 100 cases have been reported, most of which were diagnosed at splenectomy for spontaneous rupture or at autopsy [5].…”

“…In about one-third of reported cases hemorrhagic ascites is present secondary to spontaneous splenic rupture, which accelerates the disease and causes immediate danger due to hemodynamic instability [3,4,6]. Metastases occur early in the natural history of the disease and are present in about 80 % of cases [7].…”

“…Several CT appearances have been described for splenic angiosarcomas. These include a low-density mass with peripheral enhancement; homogeneous low-density mass with no contrast enhancement; high-density mass with minimal enhancement; and multiple varying-sized heterogeneously enhancing masses in an enlarged spleen [1,4,13,14]. Cystic and necrotic areas may be seen within the mass.…”

“…In our patient, the tumor showed mixed attenuation with a thin rim of irregular peripheral enhancement. Percutaneous needle biopsy of the primary lesion is not recommended due to the increased risk of bleeding complications [4].…”

Splenic hemangiosarcoma with massive calcification

“…On CT scans, splenic hemangiosarcoma appears as a heterogeneous low-density mass with peripheral enhancement or a homogeneous low-density mass with no contrast enhancement [2,9]. Occasionally, areas of high CT attenuation may be seen that represent acute hemorrhage and hemosiderin deposits [2,9].…”