Overview

NCI Definition: A WHO grade ll astrocytic tumor with a relatively favorable prognosis. It is characterized by pleomorphic and lipidized cells expressing GFAP often surrounded by a reticulin network and eosinophilic granular bodies. It presents in the superficial cerebral hemispheres and involves the meninges. It typically affects children and young adults. [1]

Pleomorphic xanthoastrocytomas most frequently harbor alterations in BRAF, CDKN2B, CDKN2A, NOTCH1, and SPTA1 [2].

BRAF V600E, BRAF Mutation, BRAF Exon 15 Mutation, BRAF Codon 600 Missense, and CDKN2B Loss are the most common alterations in pleomorphic xanthoastrocytoma [2].

Significant Genes in Pleomorphic Xanthoastrocytoma

Disease Details

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.