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 [2].

Most Commonly Altered Genes in Anaplastic Pleomorphic Xanthoastrocytoma

BRAF V600E, BRAF Mutation, BRAF Exon 15 Mutation, and BRAF Codon 600 Missense 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
Parent(s)
Encapsulated 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 6. 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.