He spleen in a patient wit H Klippel- t rénaunay syn D rome

Hemangioma is the most common primary benign neoplasm of spleen and usually represents a small localized tumor. Diffuse hemangiomatosis of the spleen is a variant of hemangioma in which the splenic parenchyma is widely replaced by vascular proliferations. This condi tion may be confined to the spleen or may occur as a manifestation of systemic angiomatosis. Associations of splenic hemangiomatosis with Beckwith-Wiedeman syndrome, KlippelTrenaunay syndrome and blue rubber bleb naevus syndrome have been reported. In this paper we present the case of a patient with KlippelTrenaunay syndrome with an incidentally discovered splenomegaly caused by diffuse hemangiomatosis. This case-report emphasizes the importance of the presence of visceral vascular malformations in patients with Klippel-Trenaunay syndrome. Case presentation