The TBL1XR1 mutation rate in our series and the recurrent deletions of 3q26.32 (TBL1XR1 locus) reported in PCNSL [7], extracerebral DLBCL [15], and acute lymphoblastic leukemia [27, 28] suggest its potential role as a tumor suppressor. The gene discussed is TBL1XR1; the disease is primary central nervous system lymphoma.