Sclerosing angiomatoid nodular transformation of spleen / 中华肝胆外科杂志

ABSTRACT

Sclerosing angiomatoid nodular transformation (SANT) is a rarely encountered benign spleen lesion which has recently been gaining recognition. However,the actual pathogenesis of this disease remains unclear.SANT's remarkably characteristic appearance consists grossly of multiple well-circumscribed vascular/angiomatoid nodules of various sizes in a fibrosclerotic stroma. Immunohistochemical studies display a heterogeneous staining pattern of endothelial phenotypes in the angiomatoid nodules,with some cells resembling splenic sinusoids (CD34 -/CD31 +/CD8 + ),capillaries (CD34 +/CD31 +/CD8 - ) and small veins (CD34 -/CD31+/CD8-).Due to the rarity of this disease,it is often mistaken for splenic hamartoma,inflammatory pseudotumor,littoral cell angima or hemangioendothelioma. So far,SANT exhibits a benign clinical course because splenectomy can be curative.In this review,we outline the clinical and pathologic features of SANT and discuss its probable disease mechanism to provide a holistic overview of the disease at this time.