Myoid Angioendothelioma of the Spleen: A Rare Case Report and Literature Review

Most common tumors of the spleen are hematologic and lymphoid malignancies. Non-lymphoid and non-hematologic tumors of the spleen are very rare, the most common of which are vascular tumors. This group of tumors in the spleen is composed of heterogeneous tumors such as hemangioma, angioendothelioma (AE), littoral cell angioma, and angiosarcoma. There are several histologic forms of AE such as epithelioid AE, Kaposiform AE, and myoid AE. Among these splenic vascular tumors, myoid angioendothelioma (MAE) seems to be the least common type. It is a distinct tumor; composed of endothelium-derived tumor cells and a special type of stromal tumor cells that seems to have borderline low-grade malignant potential. Herein, we report our experience with a young woman presented with chronic abdominal pain and splenic mass that turned out to be myoid AE of spleen with an uneventful surgery and excellent recovery period. To the best of our knowledge, such an occurrence is very rare in the spleen.