The main differential diagnosis is with the malignant rhabdoid tumors (MRT), a neoplasm more common in childhood and characterized by genetic inactivation of SMARCB1 (SNF5, INI-1), a component of the SWI/SNF chromatin remodelling complex or deletions of chromosome 22q [4-6]. The gene discussed is SMARCB1; the disease is rhabdoid tumor.