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Clinicopathological features of primary diffuse large B‐cell lymphoma of the central nervous system – strong EZH2 expression implying diagnostic and therapeutic implication

2016; Wiley; Volume: 124; Issue: 12 Linguagem: Inglês

10.1111/apm.12623

1600-0463

Shuangping Guo, Qing-Xian Bai, Joseph Rohr, Yingmei Wang, Yang Liu, Kaixuan Zeng, Kangjie Yu, Xiumin Zhang, Zhe Wang,

Glioma Diagnosis and Treatment

Primary diffuse large B‐cell lymphoma of the central nervous system ( CNS DLBCL ) is a rare entity which is difficult to diagnose and treat. The histone methyltransferase EZH 2 was reported to be involved in the tumorigenesis of systemic DLBCL but has not been implicated in primary CNS DLBCL . The clinicopathological features of 33 cases of primary CNS DLBCL and expression of EZH 2 and Y641 mutation were assessed. The tumor cells of the majority cases resembled centroblasts, and intriguingly, three cases of rare anaplastic variant were observed. Immunophenotypically, 25/33 (75.8%) cases were non‐germinal center B‐cell‐like type. Several cases (10/33; 30.3%) co‐expressed BCL 2 and MYC , 6/33 (18.2%) expressed both BCL 6 and MYC , and 5/33 (15.2%) expressed BCL 2, BCL 6, and MYC . MYC expression alone and BCL 2/ MYC co‐expression were associated with poor prognosis. EZH 2 was strongly expressed in all 33 cases independent of Y641 mutation and was significantly associated with the tumor proliferative index Ki67. However, no association was found between the level of EZH 2 expression and outcomes of patients. In summary, the clinicopathological features including three rare anaplastic variant of primary CNS DLBCL are described. Strong expression of EZH 2 in all the primary CNS DLBCL and association with high proliferative index provides further information for treatment and diagnosis of this distinctive entity.