Overview

NCI Definition: A biologic subset of diffuse large B-cell lymphomas with a unique molecular signature or expression profile. It represents approximately 50% of diffuse large B-cell lymphomas, and is characterized by the expression of CD10, BCL-6, A-myb, and LMO2 genes, BCL-2 translocation, and c-REL amplification. Morphologically, the vast majority of these lymphomas are centroblastic and a small minority are immunoblastic (ratio20:1). Patients with this type of diffuse large B-cell lymphoma have a more favorable outcome, with a 5-year survival rate of 60% and a median survival of 10 years. [1]

Germinal center B-cell-like diffuse large B-cell lymphomas most frequently harbor alterations in SIN3A, PAX5, MYD88, MAP2K1, and KMT2D [2].

Most Commonly Altered Genes in Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma

SIN3A X387_splice, PAX5 Mutation, PAX5 K5I, MYD88 Mutation, and MYD88 L265P are the most common alterations in germinal center B-cell-like diffuse large B-cell lymphoma [2].

Top Alterations in Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma

Significant Genes in Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma

AFF1 +

ALK +

BCL2 +

BCL6 +

ELL +

IRF4 +

KMT2A +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

Disease Details

Synonyms
GCB-DLBCL, DLBCL Germinal Center B-Cell Type, Diffuse Large B-Cell Lymphoma Germinal Center B-Cell Type, Diffuse Large B-Cell Lymphoma with a Germinal Center B-Cell Expression Profile
Parent(s)
Diffuse Large B-Cell Lymphoma, Not Otherwise Specified
OncoTree Name
Germinal Center B-Cell Type
OncoTree Code
GCB

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.