Associated Genetic Biomarkers

Overview

NCI Definition: An aggressive, usually diffuse non-Hodgkin lymphoma composed of small to medium sized B-lymphocytes (centrocytes). Most patients present with advanced stage disease with lymphadenopathy, hepatosplenomegaly, and bone marrow involvement. The gastrointestinal tract is the most commonly affected extranodal site by this type of non-Hodgkin lymphoma. The vast majority of cases express the t(11;14)(q13;q32) resulting in the rearrangement of the BCL-1 gene and the overexpression of cyclin D1 mRNA. [1]

Mantle cell lymphomas most frequently harbor alterations in ATM, TP53, KMT2D, CCND1, and CDKN2B [2].

Most Commonly Altered Genes in Mantle Cell Lymphoma

ATM Mutation (germline), ATM Mutation, TP53 Mutation, KMT2D Mutation, and CDKN2B Loss are the most common alterations in mantle cell lymphoma [2].

Top Alterations in Mantle Cell Lymphoma

Biomarker-Directed Therapies

Significant Genes in Mantle Cell Lymphoma

ABL1 +

AFF1 +

ALK +

ASXL1 +

ATM +

ATR +

ATRX +

BAP1 +

BARD1 +

BCL2 +

BCL6 +

BCR +

BRCA1 +

BRCA2 +

BRIP1 +

C11ORF30 +

CBFB +

CCND1 +

CDK12 +

CHEK1 +

CHEK2 +

DEK +

ELL +

ERBB2 +

ERCC2 +

FANCA +

FANCB +

FANCC +

FANCD2 +

FANCE +

FANCF +

FANCG +

FANCI +

FANCL +

FANCM +

FLT3 +

HDAC1 +

HDAC2 +

HRAS +

IGH +

IKZF1 +

IRF4 +

JAK2 +

KIT +

KMT2A +

KRAS +

MDM2 +

MDM4 +

MECOM +

MLF1 +

MLH1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MRE11A +

MUTYH +

MYC +

MYH11 +

NBN +

NOTCH1 +

NPM1 +

NRAS +

NUP214 +

PALB2 +

PARP1 +

PARP2 +

PBX1 +

PML +

POLD1 +

POLE +

POLQ +

PPP2R1A +

PPP2R2A +

PRKDC +

RAD50 +

RAD51 +

RAD51B +

RAD51C +

RAD51D +

RAD54L +

RARA +

RPN1 +

RUNX1 +

RUNX1T1 +

SLX4 +

STAG2 +

STK11 +

TCF3 +

TP53 +

XRCC1 +

Disease Details

Synonyms
MCL, Mantle Zone Lymphoma
Parent(s)
Mature B-Cell Non-Hodgkin Lymphoma
OncoTree Name
Mantle Cell Lymphoma
OncoTree Code
MCL

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 4. 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.