Overview

NCI Definition: A usually indolent mature B-cell lymphoma, arising from the marginal zone of lymphoid tissues. It is characterized by the presence of small to medium sized atypical lymphocytes. It comprises three entities, according to the anatomic sites involved: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, which affects extranodal sites (most often stomach, lung, skin, and ocular adnexa); nodal marginal zone B-cell lymphoma, which affects lymph nodes without evidence of extranodal disease; and splenic marginal zone B-cell lymphoma, which affects the spleen and splenic hilar lymph nodes, bone marrow, and often the peripheral blood. [1]

Marginal zone lymphomas most frequently harbor alterations in TNFAIP3, NOTCH1, CREBBP, ARID1B, and TET2 [2].

Most Commonly Altered Genes in Marginal Zone Lymphoma

NOTCH1 Mutation, CREBBP Mutation, TNFAIP3 Loss, TET2 Mutation, and SMO Mutation are the most common alterations in marginal zone lymphoma [2].

Top Alterations in Marginal Zone Lymphoma

Biomarker-Directed Therapies

Significant Genes in Marginal Zone Lymphoma

ABL1 +

AFF1 +

BCL2 +

BCL6 +

BCR +

BIRC3 +

CBFB +

CCND1 +

DEK +

ELL +

FLT3 +

IGH +

IKZF1 +

KIT +

KMT2A +

MALT1 +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

MYH11 +

NPM1 +

NUP214 +

PBX1 +

RPN1 +

RUNX1 +

RUNX1T1 +

TCF3 +

TP53 +

Disease Details

Synonyms
Marginal Zone Lymphoma (MZL), MZL, MZBCL, Marginal Zone B-Cell Lymphoma, Marginal Zone Lymphoma(MZL)
Parent(s)
Mature B-Cell Non-Hodgkin Lymphoma
Children
Splenic Marginal Zone Lymphoma, Nodal Marginal Zone Lymphoma, and Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue
OncoTree Name
Marginal Zone Lymphoma
OncoTree Code
MZL

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.