Associated Genetic Biomarkers

Overview

NCI Definition: Rare tumors that affect the hematopoietic and lymphoid tissues. The cells of origin are the histiocytes and accessory cells. They can occur at any age and show no significant variations in geographical distribution. This category includes the histiocytic sarcoma, Langerhans cell histiocytosis, Langerhans cell sarcoma, interdigitading dendritic cell sarcoma/tumor, follicular dendritic cell sarcoma/tumor, and dendritic cell sarcoma, not otherwise specified. (WHO, 2001) [1]

Histiocytic and dendritic cell neoplasms most frequently harbor alterations in BRAF, TET2, MAP2K1, KRAS, and DNMT3A [2].

Most Commonly Altered Genes in Histiocytic and Dendritic Cell Neoplasm

BRAF Mutation, BRAF Exon 15 Mutation, BRAF Codon 600 Missense, BRAF V600E, and MAP2K1 Mutation are the most common alterations in histiocytic and dendritic cell neoplasm [2].

Top Alterations in Histiocytic and Dendritic Cell Neoplasm

Significant Genes in Histiocytic and Dendritic Cell Neoplasm

ABL1 +

AFF1 +

AKT1 +

AKT2 +

AKT3 +

ALK +

ARAF +

ARID1A +

ASXL1 +

ATM +

ATR +

ATRX +

BARD1 +

BCR +

BRAF +

BRCA1 +

BRCA2 +

BRIP1 +

C11ORF30 +

CCND1 +

CCND2 +

CCND3 +

CCNE1 +

CCNE2 +

CDK12 +

CDK2 +

CDK4 +

CDK6 +

CDKN1A +

CDKN1B +

CDKN2A +

CHEK1 +

CHEK2 +

CRKL +

DEK +

EGFR +

ELL +

ERBB2 +

ERCC2 +

ERCC3 +

ERCC4 +

ERCC5 +

ERCC6 +

EZH2 +

FANCA +

FANCB +

FANCC +

FANCD2 +

FANCE +

FANCF +

FANCG +

FANCI +

FANCL +

FANCM +

FGFR1 +

FGFR2 +

FGFR3 +

FGFR4 +

FLT3 +

HDAC1 +

HDAC2 +

HRAS +

IRS2 +

KIT +

KMT2A +

KRAS +

MAP2K1 +

MAP2K2 +

MAP2K4 +

MAP3K1 +

MAPK1 +

MDM2 +

MDM4 +

MECOM +

MET +

MLF1 +

MLH1 +

MLH3 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MRE11A +

MSH2 +

MSH3 +

MSH6 +

MTOR +

MUTYH +

NBN +

NF1 +

NPM1 +

NRAS +

NTRK1 +

NTRK2 +

NTRK3 +

NUP214 +

PALB2 +

PARP1 +

PARP2 +

PDGFRA +

PIK3CA +

PIK3CG +

PIK3R1 +

PIK3R2 +

PMS1 +

PMS2 +

POLE +

PPP2R1A +

PPP2R2A +

PTEN +

RAD50 +

RAD51 +

RAD51B +

RAD51C +

RAD51D +

RAD54L +

RAF1 +

RB1 +

RICTOR +

RNF43 +

ROS1 +

RPN1 +

RPTOR +

RUNX1 +

SLX4 +

SMARCA4 +

SMARCB1 +

SRC +

STAG2 +

STK11 +

TP53 +

TSC1 +

TSC2 +

XRCC1 +

Disease Details

Synonyms
Histiocytic and Dendritic Cell Neoplasms, Histiocytic and Dendritic Cell Tumors
Parent(s)
Hematopoietic and Lymphoid Cell Neoplasm
Children
Histiocytosis, Non-langerhans-cell Histiocytosis, Erdheim-Chester Disease, Langerhans Cell Histiocytosis, Indeterminate Dendritic Cell Tumor, Follicular Dendritic Cell Sarcoma, Histiocytic And Dendritic Cell Cancer, and Blastic Plasmacytoid Dendritic Cell Neoplasm
OncoTree Name
Histiocytic and Dendritic Cell Neoplasms
OncoTree Code
HDCN

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.