Sclerosing Angiomatoid Nodular Transformation of the spleen in a four-year-old with anemia

Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen, first reported as a distinct histopathological entity in 2004, is exceedingly rare in children. The clinical progression and pathophysiologic features of this disease are poorly described in the literature. We performed a literature review of reported cases of SANT. Out of 258 reported cases, only five were in children. We present a sixth case of SANT in a four-year-old boy who presented with chronic anemia and elevated inflammatory markers. The relative variability in case presentation and the clinical concern for malignancy, warrant further disease characterization and discussion of management.