Overview

NCI Definition: A unique group of rare tumors of the central nervous system that affect mainly children and adolescents. Their morphologic and biologic profile corresponds to that of homologous germ cell tumors that arise in the gonads and in other extragonadal sites. Representative examples include: germinoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma, and teratoma. [1]

Central nervous system germ cell tumors most frequently harbor alterations in KRAS, CDKN1B, TP53, H3F3C, and CCND2 [2].

Most Commonly Altered Genes in Central Nervous System Germ Cell Tumor

CDKN1B Amplification, KRAS Amplification, TP53 c.217-c.1178 Missense, TP53 Mutation, and TP53 Missense are the most common alterations in central nervous system germ cell tumor [2].

Top Alterations in Central Nervous System Germ Cell Tumor

Disease Details

Synonyms
Germ Cell Neoplasm of Central Nervous System, Germ Cell Tumor of the CNS, Central Nervous System Germ Cell Neoplasm, Germ Cell Tumor of CNS, CNS Germ Cell Tumor, Germ Cell Neoplasm of the CNS, Germ Cell Tumor of Central Nervous System, Germ Cell Neoplasm of CNS, Germ Cell Tumor of the Central Nervous System, CNS Germ Cell Neoplasm, Germ Cell Neoplasm of the Central Nervous System
Parent(s)
Extragonadal Germ Cell Tumor
Children
Adult Central Nervous System Germ Cell Tumor, Central Nervous System Adult Germ Cell Tumor, Central Nervous System Childhood Germ Cell Tumor, Malignant Central Nervous System Germ Cell Tumor, Childhood Central Nervous System Germ Cell Tumor, Pineal Region Germ Cell Tumor, and Central Nervous System Teratoma
OncoTree Name
Germ Cell Tumor, Brain
OncoTree Code
BGCT

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.