Splenic leiomyoma: an uncommon localization

Muscle cell tumors are very uncommon in the spleen as they are in ataxia-telangiectasia. The increased frequency of chromosome breaks probably because of a defect in DNA repair favors the development of benign or malignant tumors in the patient. A case of leiomyoma of the spleen in an 8-year-old boy with ataxia-telangiectasia is reported. The literature on the splenic masses and the localization of leiomyoma is reviewed.