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Primitive Neuroectodermal Tumor

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Associated Genetic Biomarkers

Overview

NCI Definition: A malignant neoplasm that originates in the neuroectoderm. The neuroectoderm constitutes the portion of the ectoderm of the early embryo that gives rise to the central and peripheral nervous systems and includes some glial cell precursors. [1]

Primitive neuroectodermal tumors most frequently harbor alterations in MYCN, ALK, ATRX, CD79B, and SOX9 [2].

Most Commonly Altered Genes in Primitive Neuroectodermal Tumor

MYCN Amplification, ALK Mutation, ALK Missense, ATRX Mutation, and ALK Codon 1174 Missense are the most common alterations in primitive neuroectodermal tumor [2].

Top Alterations in Primitive Neuroectodermal Tumor

Clinical Trials

View Clinical Trials for Primitive Neuroectodermal Tumor

Significant Genes in Primitive Neuroectodermal Tumor

EGFR +

ERBB2 +

ERBB4 +

EWSR1 +

FUS +

H3F3A +

Disease Details

Synonyms
High Grade Neuroectodermal Tumor, PNET, Primitive Neuroectodermal Neoplasm, Neuroectodermal Tumor, Neuroepithelioma, High-Grade Neuroectodermal Tumor, Neuroectodermal Neoplasm
Parent(s)
Neuroepithelial Neoplasm
Children
Neuroblastic Tumor and Peripheral Primitive Neuroectodermal Tumor
OncoTree Name
Primitive Neuroectodermal Tumor
OncoTree Code
PNET

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.