PMID- 32889391
OWN - NLM
STAT- MEDLINE
DCOM- 20210713
LR  - 20210713
IS  - 1532-8198 (Electronic)
IS  - 1092-9134 (Linking)
VI  - 48
DP  - 2020 Oct
TI  - Analysis of primary central nervous system large B-cell lymphoma in the era of 
      high-grade B-cell lymphoma: Detection of two cases with MYC and BCL6 
      rearrangements in a cohort of 12 cases.
PG  - 151610
LID - S1092-9134(20)30156-8 [pii]
LID - 10.1016/j.anndiagpath.2020.151610 [doi]
AB  - High-grade diffuse large B-cell lymphoma (HG-DLBCL) refers to DLBCL with MYC and 
      BCL2 and/or BCL6 rearrangements (double-hit or triple-hit DLBCL) that exhibits 
      poor prognosis. Double-expressor DLBCL (c-myc+/bcl-2+) has intermediate prognosis 
      when compared to HG-DLBCL. Primary central nervous system lymphoma (PCNSL) has 
      distinct pathophysiology (frequent non-germinal center-like subtype and 
      double-expressor) and has worse prognosis than systemic DLBCL. By fluorescence in 
      situ hybridization (FISH), 25-30% of PCNSLs harbor BCL6 abnormalities with rare 
      alterations in MYC, BCL2, double-hit or triple-hit events. We describe the 
      clinicopathologic features and status of MYC, BCL2 and BCL6 in 12 PCNSLs (7 
      women, 5 men; median age 63 years; range: 28-79). Six cases showed focal 
      starry-sky pattern. Immunohistochemically, all (100%) were of non-germinal 
      center-like subtype, and 8/10 (80%) cases were double-expressors. Ki-67 ranged 
      from 70 to 100%. FISH was positive in 9/12 (75%) cases: 4 (33%) harbored a BCL6 
      rearrangement, 3 (25%) had a gain of BCL2, 2 (17%) cases each had a gain of BCL6 
      and gain of IGH, and gain of MYC and deletion of MYC were observed in 1 case each 
      (8%). Two (16%) cases were MYC/BCL6 double-hit PCNSLs. No MYC/BCL2 or triple-hit 
      cases were identified. Eleven (92%) patients received chemotherapy and one also 
      received whole brain radiation. The median time of follow-up was 4.4 months 
      (range, 0.3-40.3). Seven (58%) patients are alive, 4 (33%) have died, and 1 (8%) 
      had no follow-up. Five alive patients are in remission, including one MYC/BCL6 
      double-hit PCNSL. Our results add two new cases of rare double-hit PCNSL to the 
      literature.
CI  - Copyright (c) 2020 Elsevier Inc. All rights reserved.
FAU - Pina-Oviedo, Sergio
AU  - Pina-Oviedo S
AD  - Department of Pathology, University of Arkansas for Medical Sciences, Little 
      Rock, AR, United States of America.
FAU - Bellamy, William T
AU  - Bellamy WT
AD  - Department of Pathology, University of Arkansas for Medical Sciences, Little 
      Rock, AR, United States of America.
FAU - Gokden, Murat
AU  - Gokden M
AD  - Department of Pathology, University of Arkansas for Medical Sciences, Little 
      Rock, AR, United States of America. Electronic address: mgokden@uams.edu.
LA  - eng
PT  - Journal Article
DEP - 20200825
PL  - United States
TA  - Ann Diagn Pathol
JT  - Annals of diagnostic pathology
JID - 9800503
RN  - 0 (BCL6 protein, human)
RN  - 0 (Ki-67 Antigen)
RN  - 0 (Proto-Oncogene Proteins c-bcl-6)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Case-Control Studies
MH  - Central Nervous System/*pathology/radiation effects
MH  - Drug Therapy/methods
MH  - Female
MH  - Follow-Up Studies
MH  - Genes, myc/*genetics
MH  - Humans
MH  - In Situ Hybridization, Fluorescence/methods
MH  - Ki-67 Antigen/metabolism
MH  - Lymphoma, Large B-Cell, Diffuse/classification/*genetics/pathology/therapy
MH  - Male
MH  - Middle Aged
MH  - Neoplasm Grading/methods
MH  - Prognosis
MH  - Proto-Oncogene Proteins c-bcl-6/*genetics
MH  - Radiotherapy/methods
MH  - Treatment Outcome
OTO - NOTNLM
OT  - BCL6
OT  - Double-hit lymphoma
OT  - High-grade B-cell lymphoma
OT  - Primary central nervous system lymphoma
OT  - c-myc
EDAT- 2020/09/06 06:00
MHDA- 2021/07/14 06:00
CRDT- 2020/09/05 12:27
PHST- 2020/05/11 00:00 [received]
PHST- 2020/06/30 00:00 [revised]
PHST- 2020/08/19 00:00 [accepted]
PHST- 2020/09/06 06:00 [pubmed]
PHST- 2021/07/14 06:00 [medline]
PHST- 2020/09/05 12:27 [entrez]
AID - S1092-9134(20)30156-8 [pii]
AID - 10.1016/j.anndiagpath.2020.151610 [doi]
PST - ppublish
SO  - Ann Diagn Pathol. 2020 Oct;48:151610. doi: 10.1016/j.anndiagpath.2020.151610. 
      Epub 2020 Aug 25.