Overview

NCI Definition: Neoplasms that are located in the pineal gland. These neoplasms arise either from pineal parenchymal cells (pineocytes), as do pineocytomas and the more aggressive pineoblastomas, or from other cell types, as do pineal astrocytic tumors (notably pilocytic astrocytomas) and germ cell tumors. Clinical symptoms include neuro-ophthalmologic dysfunction, changes in mental status, dysfunction of the brain stem, and hypothalamic-based endocrine abnormalities. [1]

Pineal region neoplasms most frequently harbor alterations in DICER1, ATRX, EPHB1, CDK12, and CBL [2].

Most Commonly Altered Genes in Pineal Region Neoplasm

DICER1 Mutation, EPHB1 V205E, EPHB1 Mutation, CDK12 P1091S, and CDK12 Mutation are the most common alterations in pineal region neoplasm [2].

Top Alterations in Pineal Region Neoplasm

Significant Genes in Pineal Region Neoplasm

H3F3A +

Disease Details

Synonyms
Tumor of the Pineal Region, Pinealoma, Pineal Neoplasm, Pineal Area Tumor, Neoplasm of Pineal Region, Tumor of Pineal Region, Pineal Body Neoplasm, Pineal Region Tumor, Pineal Tumor, Pineal Area Neoplasm, Neoplasm of the Pineal Area, Neoplasm of the Pineal Region, Neoplasm of Pineal Area, Tumor of Pineal Area, Tumor of the Pineal Area, Pineal Body Tumor
Parent(s)
Supratentorial Neoplasm
Children
Pineal Region Germ Cell Tumor, Malignant Pineal Region Neoplasm, Benign Pineal Region Neoplasm, Papillary Tumor of the Pineal Region, Pineal Parenchymal Cell Neoplasm, and Pineal Region Meningioma
OncoTree Name
Pineal Tumor
OncoTree Code
PINT

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.