Clinicopathological features of primary diffuse large B-cell lymphoma of the central nervous system - strong EZH2 expression implying diagnostic and therapeutic implication.
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 EZH2 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 EZH2 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 BCL2 and MYC, 6/33 (18.2%) expressed both BCL6 and MYC, and 5/33 (15.2%) expressed BCL2, BCL6, and MYC. MYC expression alone and BCL2/MYC co-expression were associated with poor prognosis. EZH2 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 EZH2 expression and outcomes of patients. In summary, the clinicopathological features including three rare anaplastic variant of primary CNS DLBCL are described. Strong expression of EZH2 in all the primary CNS DLBCL and association with high proliferative index provides further information for treatment and diagnosis of this distinctive entity.
State Key Laboratory of Tumor Biology, Department of Pathology, Xi Jing Hospital, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Shuangping
S
Guo
Department of Hematology, Xi Jing Hospital, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Qingxian
Q
Bai
Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, USA.
Joseph
J
Rohr
State Key Laboratory of Tumor Biology, Department of Pathology, Xi Jing Hospital, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Yingmei
Y
Wang
State Key Laboratory of Tumor Biology, Department of Pathology, Xi Jing Hospital, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Yang
Y
Liu
State Key Laboratory of Tumor Biology, Department of Pathology, Xi Jing Hospital, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Kaixuan
K
Zeng
Student Team 1, Class 3, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Kangjie
K
Yu
State Key Laboratory of Tumor Biology, Department of Pathology, Xi Jing Hospital, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Xiumin
X
Zhang
State Key Laboratory of Tumor Biology, Department of Pathology, Xi Jing Hospital, The Fourth Military Medical University, Xi'an, Shaan Xi Province, China.
Zhe
Z
Wang
D000328:Adult; D000368:Aged; D019943:Amino Acid Substitution; D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics; D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000473:pathology*; D000071221:Enhancer of Zeste Homolog 2 Protein / Q000032:analysis* / Q000235:genetics; D005260:Female; D015870:Gene Expression; D006801:Humans; D007150:Immunohistochemistry; D019394:Ki-67 Antigen / Q000032:analysis; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D020125:Mutation, Missense; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult
D014408:Biomarkers, Tumor; D019394:Ki-67 Antigen; C498409:EZH2 protein, human; D000071221:Enhancer of Zeste Homolog 2 Protein
Primary diffuse large B-cell lymphoma of the central nervous system; anaplastic variant; clinicopathologic feature; histone methyltransferase EZH2; strong expression