Occurrence of Wilms' tumor in a child with hereditary spherocytosis

Abstract

Hereditary spherocytosis (HS) is the most frequent cause of congenitalhemolytic anemia. It is an autosomal dominant genetic disorder characterizedby cell membrane abnormalities, specifically in red blood cells. Althoughthe association between benign, borderline and malignant tumors and HS isnot clear, various tumors such as splenoma, adrenal myolipoma, pancreaticschwannoma, ganglioneuroma, extramedullary hematopoiesis, myeloproliferativedisorders, multiple myeloma, B-cell lymphoma and acute lymphoblasticleukemia have been presented in case reports concerning HS patients. Herewe describe a 6-year-old boy with HS who presented with a mass in theleft kidney. Tru-cut biopsy revealed Wilms' tumor (WT). To the best of ourknowledge, this is the first case of WT associated with HS to be reportedin the literature.