Sclerosing angiomatoid nodular transformation in the spleen A case series study and literature review

Abstract

To assess the clinicopathologic features, differential diagnosis, and pathogenesis of sclerosing angiomatoid nodular transformation (SANT) of the spleen. The clinical characteristics of 7 cases with SANT were retrospectively analyzed. Histochemical and immunohistochemical (EnVision method) examinations were performed. Moreover, quantitative assessment of IgG4 and IgG-positive cells was performed. The 7 SANT patients included 1 female and 6 males, with ages ranging from 7 to 60 years (mean 33.4 years). They showed no specific clinical manifestations. Macroscopically, the surface of the excised masses was gray-white, and vague nodularity was observed. Mass sizes ranged from 3.0 to 7.0cm (mean 5.5 cm). Microscopically, all cases were characterized based on multiple angiomatoid nodules of various sizes embedded in a fibrosclerotic stroma. The nodules were round and sometimes convoluted. Moreover, the nodules were composed of slit-like, irregularly-shaped, or slightly dilated vascular spaces lined by plump endothelial cells, and interspersed with a population of spindly or ovoid cells. Immunohistochemical examination showed a heterogeneous staining pattern, with the lining cells of small capillaries expressing CD34 and those of sinusoid-like structures expressing CD8. CD31 was stained in the lining and interspersed cells, thus resulting in a complex meshwork. Additionally, the lining cells were focally positive for CD68. Vimentin and smooth muscle antibody were expressed in all 7 cases, whereas no desmin or CD21 was detected. IgG4-positive cells accounted for 2 to 5 per high-power field (mean 3.7). Furthermore, the IgG-positive cells accounted for 2 to 8 per high-power field (mean 4.2). SANT is a rare primary benign tumor-like lesion of the spleen, and has characteristic histopathological features and immunohistochemical profiles. SANT should be distinguished from other angiomatoid tumors and tumor-like lesions. Moreover, SANT could be treated by splenectomy, with favorable prognosis. The relationship between SANT and IgG4-related sclerosing lesions remains to be clarified.