Primary central nervous system lymphoma
diseaseOn this page
Also known as central nervous system lymphomaCNS lymphomalymphoma of central nervous systemmalignant lymphomas of CNSmalignant lymphomas of the CNSmicrogliomaPCNSLprimary brain lymphomaprimary CNS lymphomaprimary lymphoma, CNS
Summary
Primary central nervous system lymphoma (MONDO:0002571) is a cancer and 183 clinical trials. Top therapeutic interventions include methotrexate, tirabrutinib, and zanubrutinib. A subtype of immune system cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: 1-9 / 1 000 000 (United States) [Orphanet-validated]
- Clinical trials: 183
Clinical features
Epidemiology
Prevalence records
2 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.44 | United States | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.26 | Sweden | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | primary central nervous system lymphoma |
| Mondo ID | MONDO:0002571 |
| EFO | EFO:1000157 |
| Orphanet | 46135 |
| DOID | DOID:3234 |
| ICD-11 | 1133193893 |
| NCIT | C9301 |
| SNOMED CT | 307649006 |
| UMLS | C0280803 |
| MedGen | 79187 |
| GARD | 0009318 |
| MedDRA | 10036685 |
| NORD | 2002 |
| Anatomy (UBERON) | UBERON:0001017 |
| Is cancer (heuristic) | yes |
Also known as: central nervous system lymphoma · CNS lymphoma · lymphoma of central nervous system · malignant lymphomas of CNS · malignant lymphomas of the CNS · microglioma · PCNSL · primary brain lymphoma · primary central nervous system lymphoma · primary CNS lymphoma · primary lymphoma, CNS
Data availability: 5 cell lines.
Disease family
An umbrella term covering 3 Mondo subtypes.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › immune system cancer › primary central nervous system lymphoma
Related subtypes (24): lymphatic system cancer, T-cell childhood acute lymphocytic leukemia, B-cell childhood acute lymphoblastic leukemia, thymus cancer, solitary plasmacytoma of chest wall, dendritic cell sarcoma, Waldeyer’s ring cancer, breast diffuse large B-cell lymphoma, colon Burkitt lymphoma, colorectal diffuse large B-cell lymphoma, gastric mantle cell lymphoma, liver diffuse large B-cell lymphoma, primary pulmonary diffuse large B-cell lymphoma, small intestinal Burkitt lymphoma, small intestinal diffuse large B-cell lymphoma, small intestinal enteropathy-associated T-cell lymphoma, thyroid gland diffuse large B-cell lymphoma, plasma cell myeloma, indolent primary cutaneous B-cell lymphoma, systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood, mast cell sarcoma, subcutaneous panniculitis-like T-cell lymphoma, bone marrow cancer, primary vitreoretinal large b-cell lymphoma
Subtypes (3): spinal cord lymphoma, cerebral lymphoma, central nervous system non-hodgkin lymphoma
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 183.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 77 |
| Not specified | 38 |
| PHASE1 | 32 |
| PHASE1/PHASE2 | 21 |
| PHASE3 | 5 |
| PHASE4 | 4 |
| PHASE2/PHASE3 | 4 |
| EARLY_PHASE1 | 2 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT05518383 | PHASE4 | RECRUITING | B-cell Mature Non-Hodgkin’s Lymphoma Treatment Protocol in Children and Adolescents 2021 |
| NCT00153530 | PHASE4 | COMPLETED | Whole Brain Irradiation in Primary Central Nervous System (CNS) Lymphoma (PCNSL) |
| NCT01960192 | PHASE4 | UNKNOWN | Treatment of Primary CNS Lymphoma |
| NCT04083066 | PHASE4 | UNKNOWN | Open Randomized Prospective Clinical Study of R-FPD Versus R-MAD Regimen in the Treatment of Primary Central Nervous System Lymphoma |
| NCT02657785 | PHASE2/PHASE3 | RECRUITING | Treatment of PCNSL With R-IDARAM and Intrathecal Immunochemotherapy |
| NCT02836158 | PHASE2/PHASE3 | RECRUITING | Therapeutic Effects of R-IDARAM and Intrathecal Immunochemotherapy on Elderly Patients With PCNSL |
| NCT04931368 | PHASE3 | ACTIVE_NOT_RECRUITING | OptiMATe: De-escalated Induction Treatment in Primary CNS Lymphoma |
| NCT06830421 | PHASE3 | RECRUITING | Adjusted High-dose Chemotherapy With Autologous Stem Cell Transplant vs. Conventional Immunochemotherapy in Elderly PCNSL Patients |
| NCT07185373 | PHASE2/PHASE3 | NOT_YET_RECRUITING | Orelabrutinib Combined With Teniposide, Rituximab and Methotrexate for Newly Diagnosed PCNSL |
| NCT02313389 | PHASE3 | UNKNOWN | Maintenance Treatment Versus Observation in Elderly Patients With PCNS Lymphoma |
| NCT03733327 | PHASE2/PHASE3 | UNKNOWN | BUCYE Conditioning Regimen for PCNSL Undergoing Auto-HSCT |
| NCT05054426 | PHASE3 | UNKNOWN | Intermediate Dose of IV MTX as CNS Prophylaxis for High Risk DLBCL |
| NCT05334238 | PHASE3 | UNKNOWN | Orelabrutinib Maintenance Therapy After ASCT in Primary Central Nervous System Lymphoma |
| NCT00863460 | PHASE2 | ACTIVE_NOT_RECRUITING | Cranial Radiotherapy or Intensive Chemotherapy With Hematopoietic Stem Cell Rescue for Primary Central Nervous System Lymphoma in Young Patients |
| NCT02952508 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of Iopofosine I-131 (CLR 131) in Select B-Cell Malignancies (CLOVER-1) With Expansion in Waldenstrom |
| NCT03277729 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | A Phase I/II Study to Evaluate the Safety of Cellular Immunotherapy Using Autologous T Cells Engineered to Express a CD20-Specific Chimeric Antigen Receptor for Patients With Relapsed or Refractory B Cell Non-Hodgkin Lymphomas |
| NCT03328078 | PHASE1/PHASE2 | RECRUITING | CA-4948-101: Open-Label, Dose Escalation and Expansion Trial of Emavusertib (CA-4948) in Relapsed or Refractory Primary Central Nervous System Lymphoma (R/R PCNSL) |
| NCT03964090 | PHASE2 | ACTIVE_NOT_RECRUITING | Temozolomide, Etoposide, Doxil, Dexamethasone, Ibrutinib, and Rituximab (TEDDI-R) in Aggressive B-cell Lymphomas With Secondary Involvement of the Central Nervous System (CNS) |
| NCT04070040 | PHASE2 | RECRUITING | Camrelizumab for Patients with Recurrent Primary Central Nervous System Lymphoma (PCNSL) |
| NCT04186520 | PHASE1/PHASE2 | RECRUITING | CAR-20/19-T Cells in Patients With Relapsed Refractory B Cell Malignancies |
| NCT04421560 | PHASE1/PHASE2 | RECRUITING | Pembrolizumab, Ibrutinib and Rituximab in PCNSL |
| NCT04548648 | PHASE2 | ACTIVE_NOT_RECRUITING | A Pilot Study of Acalabrutinib in Relapsed/Refractory Primary and Secondary CNS Lymphomas |
| NCT04627753 | PHASE2 | RECRUITING | Study of Lenalidomide/Rituximab Maintenance for Transplantation Ineligible Patients With PCNSL. |
| NCT04737889 | PHASE2 | RECRUITING | Rituximab, Lenalidomide Combined With Methotrexate and Temozolomide For Primary Central Nervous System Lymphoma |
| NCT04792489 | PHASE2 | RECRUITING | DALY II USA/ MB-CART2019.1 for DLBCL |
| NCT04906096 | PHASE2 | RECRUITING | Paxalisib (GDC-0084) In Recurrent Or Refractory PCNSL |
| NCT04906902 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Acalabrutinib in CNSL |
| NCT04947319 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of Tirabrutinib (ONO-4059) in Patients With Primary Central Nervous System Lymphoma (PROSPECT Study) |
| NCT05209620 | PHASE2 | RECRUITING | Orelabrutinib Combined With Pemetrexed For Relapsed/Refractory Central Nervous System Lymphoma |
| NCT05256641 | PHASE1/PHASE2 | RECRUITING | Acalabrutinib Maintenance for the Treatment of Patients With Large B-cell Lymphoma |
| NCT05260619 | PHASE2 | RECRUITING | Lenalidomide Maintenance Treatment in Patients With Primary Central Nervous System Lymphoma |
| NCT05347641 | PHASE2 | NOT_YET_RECRUITING | Penpulimab Combined With RMA Treatment of Primary Diagnosis of Primary Central Nervous System Lymphoma |
| NCT05351593 | PHASE1/PHASE2 | RECRUITING | Tafasitamab Plus Lenalidomide in Relapsed CNS Lymphoma |
| NCT05425654 | PHASE2 | RECRUITING | RL-MPV Followed by BBC HCT Using Autologous Stem Cells and Maintenance Therapy With Nivolumab for Newly Diagnosed PCNSL |
| NCT05485753 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | A Study of GNC-038, a Tetra-specific Antibody, in Patients With Central Nervous System Lymphoma (PCNSL) and Relapsed or Refractory Secondary Central Nervous System Lymphoma (SCNSL) |
| NCT05549284 | PHASE2 | RECRUITING | Orelabrutinib,Rituximab and Methotrexate in Newly-diagnosed Primary Central Nervous System Lymphoma(PCNSL) |
| NCT05600660 | PHASE2 | RECRUITING | Orelabrutinib,Rituximab and Methotrexate in Newly Diagnosed Primary Central Nervous System Lymphoma |
| NCT05681195 | PHASE2 | RECRUITING | Zanubrutinib With Pemetrexed to Treat Relapsed/Refractory Primary and Secondary Central Nervous System (CNS) Lymphomas |
| NCT05698147 | PHASE1/PHASE2 | RECRUITING | Selinexor in Combination With MTX+Ritu to Treat R/R CNSL |
| NCT06132737 | PHASE1/PHASE2 | RECRUITING | [90Y]Y-PTT Endoradiotherapy in CNS Lymphoma Patients |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| METHOTREXATE | 4 | 33 |
| TIRABRUTINIB | 4 | 4 |
| ZANUBRUTINIB | 4 | 4 |
| ACALABRUTINIB | 4 | 3 |
| CYCLOPHOSPHAMIDE ANHYDROUS | 4 | 3 |
| PROCARBAZINE | 4 | 3 |
| EPCORITAMAB | 4 | 2 |
| GLUCARPIDASE | 4 | 2 |
| PIRTOBRUTINIB | 4 | 2 |
| BUSULFAN | 4 | 1 |
| CYTARABINE | 4 | 1 |
| DOXORUBICIN HYDROCHLORIDE | 4 | 1 |
| ETOPOSIDE | 4 | 1 |
| FERUMOXYTOL | 4 | 1 |
| GLOFITAMAB | 4 | 1 |
| IDARUBICIN | 4 | 1 |
| IFOSFAMIDE | 4 | 1 |
| LONCASTUXIMAB TESIRINE | 4 | 1 |
| MANNITOL | 4 | 1 |
| POLATUZUMAB VEDOTIN | 4 | 1 |
| SELINEXOR | 4 | 1 |
| SODIUM THIOSULFATE | 4 | 1 |
| SORBITOL | 4 | 1 |
| TAFASITAMAB | 4 | 1 |
| TEMOZOLOMIDE | 4 | 1 |
| TENIPOSIDE | 4 | 1 |
| TISAGENLECLEUCEL | 4 | 1 |
| UBLITUXIMAB | 4 | 1 |
| VINCRISTINE | 4 | 1 |
| VISMODEGIB | 4 | 1 |
Related Atlas pages
- Drugs: Methotrexate, Tirabrutinib, Zanubrutinib, Acalabrutinib, Cyclophosphamide, Procarbazine, Epcoritamab, Glucarpidase, Pirtobrutinib, Busulfan, Cytarabine, Doxorubicin, Etoposide, Ferumoxytol, Glofitamab, Idarubicin, Ifosfamide, Loncastuximab Tesirine, Mannitol, Polatuzumab Vedotin, Selinexor, Sodium Thiosulfate, Sorbitol, Tafasitamab, Temozolomide, Teniposide, Tisagenlecleucel, Ublituximab, Vincristine, Vismodegib