Overview

NCI Definition: An astrocytic tumor of uncertain relation to pilocytic astrocytoma. It occurs predominantly in infants and young children. It is characterized by a monomorphic architectural pattern, usually associated with the absence of Rosenthal fibers and eosinophilic granular bodies. The clinical course is usually aggressive. [1]

Pilomyxoid astrocytomas most frequently harbor alterations in BRAF, KIAA1549, NF1, FGFR1, and ZNF703 [2].

Most Commonly Altered Genes in Pilomyxoid Astrocytoma

KIAA1549-BRAF Fusion, KIAA1549 Fusion, BRAF-KIAA1549 Fusion, BRAF V600E, and BRAF Mutation are the most common alterations in pilomyxoid astrocytoma [2].

Top Alterations in Pilomyxoid Astrocytoma

Significant Genes in Pilomyxoid Astrocytoma

ARAF +

BRAF +

CRKL +

FGFR1 +

FGFR2 +

FGFR3 +

HRAS +

KRAS +

MAP2K1 +

MAP2K2 +

MAP2K4 +

MAP3K1 +

MAPK1 +

MYB +

MYBL1 +

NF1 +

NF2 +

NRAS +

PTPN1 +

RAF1 +

SOS1 +

SRC +

Disease Details

Parent(s)
Encapsulated Glioma
OncoTree Name
Pilomyxoid Astrocytoma
OncoTree Code
PMA

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.