Overview

NCI Definition: A large B-cell non-Hodgkin lymphoma arising in the mediastinum. Morphologically it is characterized by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis. Response to intensive chemotherapy, with or without radiotherapy, is usually good. (WHO, 2001) [1]

Mediastinal large B-cell lymphomas most frequently harbor alterations in SOCS1, TNFAIP3, XPO1, CIITA, and STAT6 [2].

Most Commonly Altered Genes in Mediastinal Large B-Cell Lymphoma

SOCS1fs, XPO1 Mutation, XPO1 E571K, TNFAIP3fs, and STAT6 Mutation are the most common alterations in mediastinal large B-cell lymphoma [2].

Top Alterations in Mediastinal Large B-Cell Lymphoma

Significant Genes in Mediastinal Large B-Cell Lymphoma

ABL1 +

AFF1 +

ALK +

BCL2 +

BCL6 +

BCR +

CBFB +

CCND1 +

DEK +

ELL +

FLT3 +

IGH +

IRF4 +

KIT +

KMT2A +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

MYH11 +

NPM1 +

NRIP3 +

NUP214 +

RPN1 +

RUNX1 +

RUNX1T1 +

TP53 +

Disease Details

Synonyms
B-Cell Diffuse Large Cell Lymphoma of Mediastinum, B-Cell Diffuse Large Cell Lymphoma of the Mediastinum, Mediastinal (thymic) large B-cell lymphoma, PMBL, Mediastinal B-Cell Diffuse Large Cell Lymphoma, Primary Mediastinal Clear Cell Lymphoma of B-Cell Type, Mediastinal (Thymic) Large B-Cell Lymphoma, Mediastinal Diffuse Large Cell Lymphoma with Sclerosis, Primary Mediastinal Large B-Cell Lymphoma, Primary Mediastinal (Thymic) Large B-Cell Lymphoma, Mediastinal large B-cell lymphoma, PMLCL
Parent(s)
Thymic Lymphoma
OncoTree Name
Primary Mediastinal (Thymic) Large B-Cell Lymphoma
OncoTree Code
PMBL

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.