Sclerosing angiomatoid nodular transformation in a child

Abstract

Sclerosing angiomatoid nodular transformation (SANT) is a spleen-specific benign mass-forming vascular lesion. Although there have been several reports of cases in the adult population, data in the pediatric population are exceedingly rare. Herein, we present a case of SANT with hypersplenism and investigate the unique features of this disease in pediatric patients. A 14-year-old girl visited a nearby physician with fatigue that had lasted for several months and was diagnosed with severe anemia and thrombocytopenia. Computed tomography revealed marked splenomegaly and a mass on the spleen. A malignant tumor could not be ruled out by blood and imaging tests, and splenectomy was therefore performed with laparotomy. Histopathologically, hemangiomatous nodules with a mixture of multiple vascular components were found, resulting in a diagnosis of SANT. The patient recovered and experienced no recurrence of her previous symptoms in the 18 months following the operation. Accumulation of further cases is desired to improve diagnosis and treatment for children with this extremely rare condition.

Keywords

• Sclerosing angiomatoid nodular transformation
• Splenomegaly
• Pediatrics