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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.

Publication ,  Journal Article
Pina-Oviedo, S; Bellamy, WT; Gokden, M
Published in: Ann Diagn Pathol
October 2020

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.

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Published In

Ann Diagn Pathol

DOI

EISSN

1532-8198

Publication Date

October 2020

Volume

48

Start / End Page

151610

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Radiotherapy
  • Proto-Oncogene Proteins c-bcl-6
  • Prognosis
  • Pathology
  • Neoplasm Grading
  • Middle Aged
  • Male
  • Lymphoma, Large B-Cell, Diffuse
  • Ki-67 Antigen
 

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Pina-Oviedo, S., Bellamy, W. T., & Gokden, M. (2020). 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. Ann Diagn Pathol, 48, 151610. https://doi.org/10.1016/j.anndiagpath.2020.151610
Pina-Oviedo, Sergio, William T. Bellamy, and Murat Gokden. “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.Ann Diagn Pathol 48 (October 2020): 151610. https://doi.org/10.1016/j.anndiagpath.2020.151610.
Pina-Oviedo, Sergio, et al. “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.Ann Diagn Pathol, vol. 48, Oct. 2020, p. 151610. Pubmed, doi:10.1016/j.anndiagpath.2020.151610.
Journal cover image

Published In

Ann Diagn Pathol

DOI

EISSN

1532-8198

Publication Date

October 2020

Volume

48

Start / End Page

151610

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Radiotherapy
  • Proto-Oncogene Proteins c-bcl-6
  • Prognosis
  • Pathology
  • Neoplasm Grading
  • Middle Aged
  • Male
  • Lymphoma, Large B-Cell, Diffuse
  • Ki-67 Antigen