Overview

NCI Definition: A WHO grade III pleomorphic xanthoastrocytoma characterized by the presence of five or more mitoses per 10 high-power fields. Necrosis may be present. Patients have shorter survival rates when compared to those with WHO grade II pleomorphic xanthoastrocytoma. [1]

Anaplastic pleomorphic xanthoastrocytomas most frequently harbor alterations in BRAF, TP53, ZFHX3, ATRX, and ZFHX4 [2].

Most Commonly Altered Genes in Anaplastic Pleomorphic Xanthoastrocytoma

BRAF V600E, BRAF Mutation, BRAF Exon 15 Mutation, BRAF Codon 600 Missense, and ATRX Mutation are the most common alterations in anaplastic pleomorphic xanthoastrocytoma [2].

Top Alterations in Anaplastic Pleomorphic Xanthoastrocytoma

Significant Genes in Anaplastic Pleomorphic Xanthoastrocytoma

APC +

BRAF +

CDK6 +

CTDNEP1 +

CTNNB1 +

DDX3X +

GLI2 +

H3F3A +

IDH1 +

IDH2 +

KDM6A +

KMT2C +

KMT2D +

LRP1B +

MDM4 +

MYC +

MYCL +

MYCN +

OTX2 +

PPM1D +

PTCH1 +

PTEN +

PVT1 +

RELA +

SHH +

SMARCA4 +

SMO +

SNCAIP +

SUFU +

TERT +

TP53 +

YAP1 +

ZMYM3 +

Disease Details

Synonyms
APX, Pleomorphic Xanthoastrocytoma with Anaplastic Features
Parent(s)
WHO Grade III Glioma
OncoTree Name
Anaplastic Pleomorphic Xanthoastrocytoma
OncoTree Code
APXA

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.