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Disease

Primary central nervous system lymphoma

Late-stage therapeutic development
Also known as CNS lymphoma, PCNSL, central nervous system lymphoma, lymphoma of central nervous system+6 more

CNS lymphoma, PCNSL, central nervous system lymphoma, lymphoma of central nervous system, malignant lymphomas of CNS, malignant lymphomas of the CNS, microglioma, primary CNS lymphoma, primary brain lymphoma, primary lymphoma, CNS.

19
Clinical trials
6
Associated genes
2
Related proteins

What's happening now

An analyst briefing on current research, clinical, regulatory and industry activity surrounding this disease.

Executive briefingUpdating summary…Momentum: Moderate
Key developments
  • 3 clinical trials expected to report results, the earliest in Q4 2027.
  • 1 safety action from EMA issued.
Upcoming Milestones
Major developments
Upcoming trial readoutHigh impact
A Phase 3, Multi-regional, Open-label, Randomized Study of Tirabrutinib vs Rituximab and Temozolomide in Participants With Relapsed/Refractory Primary Central Nervous System Lymphoma
Results expected Q4 20272026-07-10
Upcoming trial readoutHigh impact
Optimizing MATRix as Remission Induction in PCNSL: De-escalated Induction Treatment in Newly Diagnosed Primary CNS Lymphoma - a Randomized Phase III Trial
Results expected Q2 20282026-03-18
Major safety communicationImportant
Market withdrawal — Duvelisib
Copiktra monotherapy is indicated for the treatment of adult patients with:  Relapsed or refractory chronic lymphocytic leukaemia (CLL) after at least two prior therapies.  Follicular lymphoma (FL) that is refractory to at least two prior  systemic therapies.2026-02-16
Clinical Milestones6View
Results expected 3
Recently completed 3
Regulatory Updates1View
  • 2026-02-16Market withdrawal — DuvelisibCopiktra monotherapy is indicated for the treatment of adult patients with:  Relapsed or refractory chronic lymphocytic leukaemia (CLL) after at least two prior therapies.  Follicular lymphoma (FL) that is refractory to at least two prior  systemic therapies.
Activity timeline7

Therapeutic landscape

Therapies with a regulatory footing for this condition, alongside the wider set of agents co-studied with it in the literature.

Approved & established therapies
Odronextamabapproved

Approval — Ordspono as monotherapy is indicated for the treatment of adult patients with relapsed or… (2024)

Epcoritamabapproved

Approval — Tepkinly as monotherapy is indicated for the treatment of adult patients with relapsed or… (2023)

Glofitamabapproved

Approval — Columvi in combination with gemcitabine and oxaliplatin is indicated for the treatment of… (2023)

Pirtobrutinibapproved

Approval — Jaypirca as monotherapy is indicated for the treatment of adult patients with relapsed or… (2023)

Lisocabtagene maraleucelapproved

Approval — Breyanzi is indicated for the treatment of adult patients with diffuse large B-cell lymph… (2022)

Loncastuximab tesirineapproved

Approval — Zynlonta as monotherapy is indicated for the treatment of adult patients with relapsed or… (2022)

Mosunetuzumabapproved

Approval — Lunsumio as monotherapy is indicated for the treatment of adult patients with relapsed or… (2022)

Plerixaforapproved

Approval — Adult patients Plerixafor Accord is indicated in combination with granulocyte-colony stim… (2022)

Clinical trials

14 sponsors · 2 new · 3 completed in the last 12 months (net +2)

The current development programme across all trial phases.

Clinical programme
19
All trials
6
Active
4
Late-stage
6
Completed
Late-stage studies

Age-adjusted High-dose Chemotherapy Followed by Autologous Stem Cell Transplantation or Conventional Chemotherapy With R-MP as First-line Treatment in Elderly Primary CNS Lymphoma Patients - a Randomized Phase III Trial

Phase 3 · Recruiting · University Hospital Freiburg

A Phase 3, Multi-regional, Open-label, Randomized Study of Tirabrutinib vs Rituximab and Temozolomide in Participants With Relapsed/Refractory Primary Central Nervous System Lymphoma

Phase 3 · Recruiting · Ono Pharmaceutical Co., Ltd.

Treatment of Primary CNS Lymphoma With Systemic R-IDARAM Chemotherapy and Intrathecal Immunochemotherapy

Phase 2/3 · Recruiting · Navy General Hospital, Beijing

Optimizing MATRix as Remission Induction in PCNSL: De-escalated Induction Treatment in Newly Diagnosed Primary CNS Lymphoma - a Randomized Phase III Trial

Phase 3 · Active, not recruiting · Klinikum Stuttgart
Recruiting

Age-adjusted High-dose Chemotherapy Followed by Autologous Stem Cell Transplantation or Conventional Chemotherapy With R-MP as First-line Treatment in Elderly Primary CNS Lymphoma Patients - a Randomized Phase III Trial

Phase 3 · Recruiting · University Hospital Freiburg

A Phase 3, Multi-regional, Open-label, Randomized Study of Tirabrutinib vs Rituximab and Temozolomide in Participants With Relapsed/Refractory Primary Central Nervous System Lymphoma

Phase 3 · Recruiting · Ono Pharmaceutical Co., Ltd.

Treatment of Primary CNS Lymphoma With Systemic R-IDARAM Chemotherapy and Intrathecal Immunochemotherapy

Phase 2/3 · Recruiting · Navy General Hospital, Beijing

In Treatment-Naive Patients With Primary Central Nervous System Lymphoma (PCNSL): A Multicenter, Prospective, Concurrent-Control Study of Methotrexate, , Rituximab,, Sintilimab ,Pirtobrutinib Versus Investigator-Selected Standard of Care

Phase 2 · Recruiting · Tongji Hospital

A Single-arm, Prospective, Multicenter Clinical Trial of the Combination of Thiotepa and Pomalidomide for the Treatment of Relapsed/Refractory Primary Central Nervous System Lymphoma.

Phase 2 · Recruiting · The First Affiliated Hospital with Nanjing Medical University
Recently completed

Phase IB/II Study Combining the PI3K Inhibitor Copanlisib With the BTK Inhibitor Ibrutinib in Patients With Recurrent/Refractory Primary CNS Lymphoma

Phase 1/2 · Completed · Memorial Sloan Kettering Cancer Center

A Phase I/II Trial of the Bruton's Tyrosine Kinase (BTK) Inhibitor, Ibrutinib, in Patients With Newly Diagnosed or Refractory/Recurrent Primary Central Nervous System Lymphoma (PCNSL) and Refractory/Recurrent Secondary Central Nervous System Lymphoma (SCNSL)

Phase 1/2 · Completed · Memorial Sloan Kettering Cancer Center

Phase II Maintenance Trial of Nivolumab for Newly Diagnosed PCNSL Patients With Persistent Circulating Tumor DNA in the CSF After Completion of Methotrexate-Based First-Line Induction Chemotherapy

Phase 2 · Completed · Memorial Sloan Kettering Cancer Center

Randomized Phase II Trial on Fitness- and Comorbidity- Tailored Treatment in Elderly Patients With Newly Diagnosed Primary CNS Lymphoma (FIORELLA Trial)

Phase 2 · Completed · International Extranodal Lymphoma Study Group (IELSG)

Phase 2 Trial of Pembrolizumab in Relapsed and Refractory Gray-Zone Lymphoma (GZL), Primary Central Nervous System Lymphoma (PCNSL), and Other Extranodal Diffuse Large B-cell Lymphomas

Phase 2 · Completed · National Cancer Institute (NCI)

AIDS-Related Primary Central Nervous System Lymphoma: A Phase II Pilot Study of High-Dose Intravenous Methotrexate With Rituximab Leucovorin Rescue and Highly Active Antiretroviral Therapy

Phase 2 · Completed · National Cancer Institute (NCI)

Regulatory timeline

Drug regulatory events matched to this condition by indication — EMA.

First approvals
2024emaApproval — Odronextamab· Ordspono as monotherapy is indicated for the treatment of adult patients with relapsed or refractory follicular lymphoma (r/r FL) after two or more lines of systemic therapy. Ordspono as monotherapy is indicated for the treatment of adult patients with relapsed or refractory diffuse large B-cell lymphoma (r/r DLBCL) after two or more lines of systemic therapy. source ↗
2023emaApproval — Pirtobrutinib· Jaypirca as monotherapy is indicated for the treatment of adult patients with relapsed or refractory mantle cell lymphoma (MCL) who have been previously treated with a Bruton’s tyrosine kinase (BTK) inhibitor. Jaypirca as monotherapy is indicated for the treatment of adult patients with relapsed or refractory chronic lymphocytic leukaemia (CLL) who have been previously treated with a BTK inhibitor. source ↗
2023emaApproval — Epcoritamab· Tepkinly as monotherapy is indicated for the treatment of adult patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL) after two or more lines of systemic therapy.Tepkinly as monotherapy is indicated for the treatment of adult patients with relapsed or refractory follicular lymphoma (FL) after two or more lines of systemic therapy.Tepkinly as monotherapy is indicated for the treatment of adult patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL) after two or more lines of systemic therapy.Tepkinly as monotherapy is indicated for the treatment of adult patients with relapsed or refractory follicular lymphoma (FL) after two or more lines of systemic therapy.  source ↗
2023emaApproval — Glofitamab· Columvi in combination with gemcitabine and oxaliplatin is indicated for the treatment of adult patients with relapsed or refractory diffuse large B-cell lymphoma not otherwise specified (DLBCL NOS) who are ineligible for autologous stem cell transplant (ASCT).Columvi as monotherapy is indicated for the treatment of adult patients with relapsed or refractory diffuse large B cell lymphoma (DLBCL), after two or more lines of systemic therapy. source ↗
2022emaApproval — Loncastuximab tesirine· Zynlonta as monotherapy is indicated for the treatment of adult patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL) and high-grade B-cell lymphoma (HGBL), after two or more lines of systemic therapy. source ↗
2022emaApproval — Plerixafor· Adult patients Plerixafor Accord is indicated in combination with granulocyte-colony stimulating factor (G-CSF) to enhance mobilisation of haematopoietic stem cells to the peripheral blood for collection and subsequent autologous transplantation in adult patients with lymphoma or multiple myeloma whose cells mobilise poorly (see section 4.2). Paediatric patients (1 to less than 18 years) Plerixafor Accord is indicated in combination with G-CSF to enhance mobilisation of haematopoietic stem cells to the peripheral blood for collection and subsequent autologous transplantation in children with lymphoma or solid malignant tumours, either:- pre-emptively, when circulating stem cell count on the predicted day of collection after adequate mobilization with G-CSF (with or without chemotherapy) is expected to be insufficient with regards to desired hematopoietic stem cells yield, or- who previously failed to collect sufficient haematopoietic stem cells (see section 4.2). source ↗
2022emaApproval — Mosunetuzumab· Lunsumio as monotherapy is indicated for the treatment of adult patients with relapsed or refractory follicular lymphoma (FL) who have received at least two prior systemic therapies. source ↗
2022emaApproval — Lisocabtagene maraleucel· Breyanzi is indicated for the treatment of adult patients with diffuse large B-cell lymphoma (DLBCL), high grade B-cell lymphoma (HGBCL), primary mediastinal large B-cell lymphoma (PMBCL) and follicular lymphoma grade 3B (FL3B), who relapsed within 12 months from completion of, or are refractory to, first-line chemoimmunotherapy.   Breyanzi is indicated for the treatment of adult patients with relapsed or refractory DLBCL, PMBCL and FL3B, after two or more lines of systemic therapy.  Breyanzi is indicated for the treatment of adult patients with relapsed or refractory follicular lymphoma (FL) after two or more lines of systemic therapy.   Breyanzi is indicated for the treatment of adult patients with relapsed or refractory mantle cell lymphoma (MCL) after at least two lines of systemic therapy including a Bruton’s tyrosine kinase (BTK) inhibitor.  source ↗
Safety updates
2026emaMarket withdrawal — Duvelisib· Copiktra monotherapy is indicated for the treatment of adult patients with:  Relapsed or refractory chronic lymphocytic leukaemia (CLL) after at least two prior therapies.  Follicular lymphoma (FL) that is refractory to at least two prior  systemic therapies. source ↗
2025emaMarket withdrawal — Bortezomib· Bortezomib Hospira as monotherapy or in combination with pegylated liposomal doxorubicin or dexamethasone is indicated for the treatment of adult patients with progressive multiple myeloma who have received at least 1 prior therapy and who have already undergone or are unsuitable for haematopoietic stem cell transplantation. Bortezomib Hospira in combination with melphalan and prednisone is indicated for the treatment of adult patients with previously untreated multiple myeloma who are not eligible for high-dose chemotherapy with haematopoietic stem cell transplantation. Bortezomib Hospira in combination with dexamethasone, or with dexamethasone and thalidomide, is indicated for the induction treatment of adult patients with previously untreated multiple myeloma who are eligible for high-dose chemotherapy with haematopoietic stem cell transplantation. Bortezomib Hospira in combination with rituximab, cyclophosphamide, doxorubicin and prednisone is indicated for the treatment of adult patients with previously untreated mantle cell lymphoma who are unsuitable for haematopoietic stem cell transplantation. source ↗
2024emaMarket withdrawal — Ibritumomab tiuxetan· Zevalin is indicated in adults. [90Y]-radiolabelled Zevalin is indicated as consolidation therapy after remission induction in previously untreated patients with follicular lymphoma. The benefit of Zevalin following rituximab in combination with chemotherapy has not been established. [90Y]-radiolabelled Zevalin is indicated for the treatment of adult patients with rituximab relapsedorrefractory CD20+ follicular B-cell non-Hodgkin's lymphoma (NHL). source ↗

European Medicines Agency (CC BY 4.0). Events are matched to this condition by drug indication text — approvals/updates for drugs indicated for it, not disease-specific acts.

Associated genes

6 matches

Genes associated with this disease in the canonical knowledge graph (Open Targets evidence). Number shows the association score.

MYD880.39PIM10.38CD79B0.38BTG10.38PRDM10.37SAMD140.02

Disease biology

2 matches

Proteins whose encoding gene is associated with this disease, reached through the canonical gene→disease graph. Number shows the gene's association score.

B-cell antigen receptor complex-associated protein beta chainvia CD79B0.38PR domain zinc finger protein 1via PRDM10.37

Reference

Authoritative identity, definition & identifiers.

Synonyms

CNS lymphoma, PCNSL, central nervous system lymphoma, lymphoma of central nervous system, malignant lymphomas of CNS, malignant lymphomas of the CNS, microglioma, primary CNS lymphoma, primary brain lymphoma, primary lymphoma, CNS

References & data sources
  • Disease identity & definition — NLM Medical Subject Headings (MeSH), public domain
  • Clinical trials — ClinicalTrials.gov (U.S. National Library of Medicine)
  • Related entities are derived from literature co-mention (studied together) — associative, not causal.