Overview

NCI Definition: A neoplasm of follicle centre B cells which has at least a partial follicular pattern. Follicular lymphomas comprise about 35% of adult non-Hodgkin lymphomas in the U.S. and 22% worldwide. Most patients have widespread disease at diagnosis. Morphologically, follicular lymphomas are classified as Grade 1, Grade 2, and Grade 3, depending on the percentage of the large lymphocytes present. The vast majority of cases (70-95%) express the BCL-2 rearrangement [t(14;18)]. Histological grade correlates with prognosis. Grades 1 and 2 follicular lymphomas are indolent and grade 3 is more aggressive (adapted from WHO, 2001). [1]

Follicular lymphomas most frequently harbor alterations in KMT2D, CREBBP, BCL2, TNFRSF14, and EZH2 [2].

Most Commonly Altered Genes in Follicular Lymphoma

CREBBP Mutation, KMT2D Mutation, KMT2fs, BCL2 Mutation, and EZH2 c.1864-c.2195 Missense are the most common alterations in follicular lymphoma [2].

Top Alterations in Follicular Lymphoma

Biomarker-Directed Therapies

Significant Genes in Follicular Lymphoma

ABL1 +

AFF1 +

ALK +

ARID1A +

BCL2 +

BCL6 +

BCR +

CBFB +

CCND1 +

CREBBP +

DEK +

ELL +

EP300 +

EZH2 +

FLT3 +

IGH +

IKZF1 +

IRF4 +

KIT +

KMT2A +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

MYCN +

MYH11 +

NOTCH1 +

NPM1 +

NUP214 +

PBX1 +

RPN1 +

RUNX1 +

RUNX1T1 +

TCF3 +

TP53 +

Disease Details

Synonyms
Follicular Non-Hodgkin's Lymphoma, Follicular Centre Cell Lymphoma, Lymphoma, Follicular Centre Cell, Follicular Non-Hodgkin Lymphoma, LYMPHOMA, FOLLICULAR, MALIGNANT, Follicle Center Lymphoma
Parent(s)
Mature B-Cell Non-Hodgkin Lymphoma
Children
Diffuse Follicular Lymphoma, Grade 2 Follicular Lymphoma, Grade 3 Follicular Lymphoma, Grade 1 Follicular Lymphoma, Duodenal-Type Follicular Lymphoma, Primary Cutaneous Follicle Center Lymphoma, and Pediatric-Type Follicular Lymphoma
OncoTree Name
Follicular Lymphoma
OncoTree Code
FL

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.