NCI Definition: A non-Hodgkin or Hodgkin lymphoma that arises in the brain or spinal cord as a primary lesion. There is no evidence of lymphoma outside the central nervous system at the time of diagnosis. [1]

Central nervous system lymphomas most frequently harbor alterations in MYD88 and CDKN2A [2].

Most Commonly Altered Genes in Central Nervous System Lymphoma

MYD88 Mutation are the most common alterations in central nervous system lymphoma [2].

Top Alterations in Central Nervous System Lymphoma

Significant Genes in Central Nervous System Lymphoma


BRD3 +

BRD4 +



Disease Details

PCNSL, CNS Lymphoma, Primary CNS Lymphoma, Malignant Lymphomas of CNS, Primary Central Nervous System Lymphoma, Malignant Lymphomas of the CNS
Central Nervous System Hematopoietic Neoplasm
Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System, Primary Central Nervous System Lymphoma, Spinal Cord Lymphoma, and Cerebral Lymphoma
OncoTree Name
Malignant Lymphoma
OncoTree Code


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 6. 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.