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.
Comprehensive, easy-to-understand information about this condition
How we create this content →The documentation on primary central nervous system lymphoma is limited due to its rarity, affecting fewer than 10,000 people in the U.S. annually. This rarity restricts the scope of systematic clinical studies and comprehensive phenotype documentation. Additionally, the complexity of the condition and its overlap with other neurological disorders further complicate characterization.
To navigate primary central nervous system lymphoma, consider consulting a neuro-oncologist who specializes in brain tumors and lymphomas. Engaging with a genetic counselor may also be beneficial, especially given the uncertain genetic factors associated with the condition. While there are no specific patient organizations identified, resources like GARD (rarediseases.info.nih.gov) can provide additional information and support. Participation in clinical trials may also offer access to new treatment options.
Currently, there are several orphan drugs in development for primary central nervous system lymphoma, including (2S,5S)-5-(4-amino-5-(4-(2,3-difluorophenoxy)phenyl)imidazo[5,1-f][1,2,4]triazin-7-yl)tetrahydro-2H-pyran-2-yl)methanol and tirabrutinib. There are also 125 active clinical trials exploring various treatment options. For more information on these trials, visit ClinicalTrials.gov at https://clinicaltrials.gov/search?cond=primary%20central%20nervous%20system%20lymphoma.
Actionable guidance for navigating care for primary central nervous system lymphoma
To navigate primary central nervous system lymphoma, consider consulting a neuro-oncologist who specializes in brain tumors and lymphomas. Engaging with a genetic counselor may also be beneficial, especially given the uncertain genetic factors associated with the condition. While there are no specific patient organizations identified, resources like GARD (rarediseases.info.nih.gov) can provide additional information and support. Participation in clinical trials may also offer access to new treatment options.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
The documentation on primary central nervous system lymphoma is limited due to its rarity, affecting fewer than 10,000 people in the U.S. annually. This rarity restricts the scope of systematic clinical studies and comprehensive phenotype documentation. Additionally, the complexity of the condition and its overlap with other neurological disorders further complicate characterization.
To navigate primary central nervous system lymphoma, consider consulting a neuro-oncologist who specializes in brain tumors and lymphomas. Engaging with a genetic counselor may also be beneficial, especially given the uncertain genetic factors associated with the condition. While there are no specific patient organizations identified, resources like GARD (rarediseases.info.nih.gov) can provide additional information and support. Participation in clinical trials may also offer access to new treatment options.
Currently, there are several orphan drugs in development for primary central nervous system lymphoma, including (2S,5S)-5-(4-amino-5-(4-(2,3-difluorophenoxy)phenyl)imidazo[5,1-f][1,2,4]triazin-7-yl)tetrahydro-2H-pyran-2-yl)methanol and tirabrutinib. There are also 125 active clinical trials exploring various treatment options. For more information on these trials, visit ClinicalTrials.gov at https://clinicaltrials.gov/search?cond=primary%20central%20nervous%20system%20lymphoma.
Actionable guidance for navigating care for primary central nervous system lymphoma
To navigate primary central nervous system lymphoma, consider consulting a neuro-oncologist who specializes in brain tumors and lymphomas. Engaging with a genetic counselor may also be beneficial, especially given the uncertain genetic factors associated with the condition. While there are no specific patient organizations identified, resources like GARD (rarediseases.info.nih.gov) can provide additional information and support. Participation in clinical trials may also offer access to new treatment options.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
The documentation on primary central nervous system lymphoma is limited due to its rarity, affecting fewer than 10,000 people in the U.S. annually. This rarity restricts the scope of systematic clinical studies and comprehensive phenotype documentation. Additionally, the complexity of the condition and its overlap with other neurological disorders further complicate characterization.
To navigate primary central nervous system lymphoma, consider consulting a neuro-oncologist who specializes in brain tumors and lymphomas. Engaging with a genetic counselor may also be beneficial, especially given the uncertain genetic factors associated with the condition. While there are no specific patient organizations identified, resources like GARD (rarediseases.info.nih.gov) can provide additional information and support. Participation in clinical trials may also offer access to new treatment options.
Currently, there are several orphan drugs in development for primary central nervous system lymphoma, including (2S,5S)-5-(4-amino-5-(4-(2,3-difluorophenoxy)phenyl)imidazo[5,1-f][1,2,4]triazin-7-yl)tetrahydro-2H-pyran-2-yl)methanol and tirabrutinib. There are also 125 active clinical trials exploring various treatment options. For more information on these trials, visit ClinicalTrials.gov at https://clinicaltrials.gov/search?cond=primary%20central%20nervous%20system%20lymphoma.
Actionable guidance for navigating care for primary central nervous system lymphoma
To navigate primary central nervous system lymphoma, consider consulting a neuro-oncologist who specializes in brain tumors and lymphomas. Engaging with a genetic counselor may also be beneficial, especially given the uncertain genetic factors associated with the condition. While there are no specific patient organizations identified, resources like GARD (rarediseases.info.nih.gov) can provide additional information and support. Participation in clinical trials may also offer access to new treatment options.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Clinical profile data for this condition is not yet available. Phenotype information may still be loading below.
Research studies investigating treatments and therapies for this condition.
Active Trials
Total Trials
Data from ClinicalTrials.gov Jan 30, 2026
Consider asking your healthcare providers these condition-specific questions
European rare disease database
Genetic and Rare Diseases Info Center
AI-Generated Content: This summary was generated using AI. Content has been fact-checked. Always consult with qualified healthcare providers for medical guidance.
Kisho delivers this disease record via API, including phenotypes (HPO), genes, orphan drug designations, screening status, and PAG mapping, with version history and governance.