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 TP53, SQSTM1, SETBP1, CDKN2B, and XPO1 [2].

Most Commonly Altered Genes in Primitive Neuroectodermal Tumor

TP53 c.217-c.1178 Missense, TP53 c.142-c.212 Missense, TP53 c.1-c.137 Missense, TP53 Mutation, and TP53 Missense are the most common alterations in primitive neuroectodermal tumor [2].

Top Alterations in Primitive Neuroectodermal Tumor

Disease Details

Synonyms
Neuroepithelioma, Neuroectodermal Tumor, Primitive Neuroectodermal Neoplasm, PNET, Neuroectodermal Neoplasm
Parent(s)
Embryonal Neoplasm
Children
Melanotic Neuroectodermal Tumor, Peripheral Primitive Neuroectodermal Tumor, and Neuroblastic 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 4. 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.