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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
Latest activity
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Recent clinical, regulatory, research and industry developments relating to this disease.

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
  • 4 clinical trials expected to report results, the earliest in Q4 2026.
Upcoming Milestones
Clinical Milestones7View
Results expected 4
Recently completed 3
Regulatory Updates1View
  • 2026-02-16Product authorisation ended — 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 timeline8

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
Duvelisibapproved

Product authorisation ended — Copiktra monotherapy is indicated for the treatment of adult patients with:  Relapsed or… (2026)

Bortezomibapproved

Product authorisation ended — Bortezomib Hospira as monotherapy or in combination with pegylated liposomal doxorubicin… (2025)

Ibritumomab tiuxetanapproved

Product authorisation ended — Zevalin is indicated in adults. [90Y]-radiolabelled Zevalin is indicated as consolidation… (2024)

Odronextamabapproved

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

Epcoritamabapproved

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

Glofitamabapproved

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

Pirtobrutinibapproved

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

Lisocabtagene maraleucelapproved

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

Loncastuximab tesirineapproved

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

Mosunetuzumabapproved

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

Plerixaforapproved

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

Lenalidomideapproved

Generic approval — Multiple myelomaLenalidomide Krka as monotherapy is indicated for the maintenance treatme… (2021)

Tafasitamabapproved

Conditional marketing authorisation — Minjuvi is indicated in combination with lenalidomide followed by Minjuvi monotherapy for… (2021)

Zanubrutinibapproved

Original application approval — Brukinsa as monotherapy is indicated for the treatment of adult patients with Walden… (2021)

Acalabrutinibapproved

Original application approval — Calquence as monotherapy or in combination with obinutuzumab is indicated for the treatme… (2020)

Dimethyl fumarateapproved

Generic approval — Multiple myeloma Lenalidomide Mylan as monotherapy is indicated for the maintenance treat… (2020)

Melphalan hydrochlorideapproved

Original application approval — High-dose of Phelinun used alone or in combination with other cytotoxic medicinal product… (2020)

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 Phase 2 Study of Paxalisib (GDC-0084) in Recurrent or Refractory Primary Central Nervous System Lymphoma (PCNSL)

Phase 2 · Recruiting · Lakshmi Nayak, MD
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
2024emaConditional marketing authorisation — 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 ↗
2023emaConditional marketing authorisation — 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 ↗
2023emaConditional marketing authorisation — 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 ↗
2022emaConditional marketing authorisation — 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 ↗
2022emaConditional marketing authorisation — 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 ↗
2021emaConditional marketing authorisation — Tafasitamab· Minjuvi is indicated in combination with lenalidomide followed by Minjuvi monotherapy for the treatment of adult patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL) who are not eligible for autologous stem cell transplant (ASCT). source ↗
Product approvals and authorisations
2023emaOriginal application approval — 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 ↗
2022emaGeneric approval — 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 ↗
2022emaOriginal application approval — 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 ↗
2021emaOriginal application approval — Zanubrutinib· Brukinsa as monotherapy is indicated for the treatment of adult patients with Waldenström’s macroglobulinaemia (WM) who have received at least one prior therapy, or in first line treatment for patients unsuitable for chemo-immunotherapy. Brukinsa as monotherapy is indicated for the treatment of adult patients with marginal zone lymphoma (MZL) who have received at least one prior anti-CD20-based therapy. Brukinsa as monotherapy is indicated for the treatment of adult patients with chronic lymphocytic leukemia (CLL). Brukinsa in combination with obinutuzumab is indicated for the treatment of adult patients with refractory or relapsed follicular lymphoma (FL) who have received at least two prior systemic therapies. source ↗
2021emaGeneric approval — Lenalidomide· Multiple myelomaLenalidomide Krka as monotherapy is indicated for the maintenance treatment of adult patients with newly diagnosed multiple myeloma who have undergone autologous stem cell transplantation. Lenalidomide Krka as combination therapy with dexamethasone, or bortezomib and dexamethasone, or melphalan and prednisone (see section 4.2) is indicated for the treatment of adult patients with previously untreated multiple myeloma who are not eligible for transplant. Lenalidomide Krka in combination with dexamethasone is indicated for the treatment of multiple myeloma in adult patients who have received at least one prior therapy. Myelodysplastic syndromesLenalidomide Krka as monotherapy is indicated for the treatment of adult patients with transfusion-dependent anemia due to low- or intermediate-1-risk myelodysplastic syndromes associated with an isolated deletion 5q cytogenetic abnormality when other therapeutic options are insufficient or inadequate.Mantle cell lymphomaLenalidomide Krka as monotherapy is indicated for the treatment of adult patients with relapsed or refractory mantle cell lymphoma (see sections 4.4 and 5.1). Follicular lymphomaLenalidomide Krka in combination with rituximab (anti-CD20 antibody) is indicated for the treatment of adult patients with previously treated follicular lymphoma (Grade 1 – 3a). Multiple myelomaLenalidomide Krka as monotherapy is indicated for the maintenance treatment of adult patients with newly diagnosed multiple myeloma who have undergone autologous stem cell transplantation. Lenalidomide Krka as combination therapy with dexamethasone, or bortezomib and dexamethasone, or melphalan and prednisone (see section 4.2) is indicated for the treatment of adult patients with previously untreated multiple myeloma who are not eligible for transplant. Lenalidomide Krka in combination with dexamethasone is indicated for the treatment of multiple myeloma in adult patients who have received at least one prior therapy. Myelodysplastic syndromesLenalidomide Krka as monotherapy is indicated for the treatment of adult patients with transfusion-dependent anemia due to low- or intermediate-1-risk myelodysplastic syndromes associated with an isolated deletion 5q cytogenetic abnormality when other therapeutic options are insufficient or inadequate. Mantle cell lymphomaLenalidomide Krka as monotherapy is indicated for the treatment of adult patients with relapsed or refractory mantle cell lymphoma (see sections 4.4 and 5.1). Follicular lymphomaLenalidomide Krka in combination with rituximab (anti-CD20 antibody) is indicated for the treatment of adult patients with previously treated follicular lymphoma (Grade 1 – 3a). source ↗
2020emaGeneric approval — Dimethyl fumarate· Multiple myeloma Lenalidomide Mylan as monotherapy is indicated for the maintenance treatment of adult patients with newly diagnosed multiple myeloma who have undergone autologous stem cell transplantation. Lenalidomide Mylan as combination therapy with dexamethasone, or bortezomib and dexamethasone, or melphalan and prednisone is indicated for the treatment of adult patients with previously untreated multiple myeloma who are not eligible for transplant. Lenalidomide Mylan in combination with dexamethasone is indicated for the treatment of multiple myeloma in adult patients who have received at least one prior therapy. Follicular lymphoma Lenalidomide Mylan in combination with rituximab (anti-CD20 antibody) is indicated for the treatment of adult patients with previously treated follicular lymphoma (Grade 1-3a). source ↗
2020emaOriginal application approval — Melphalan hydrochloride· High-dose of Phelinun used alone or in combination with other cytotoxic medicinal products and/or total body irradiation is indicated in the treatment of: multiple myeloma, malignant lymphoma (Hodgkin, non-Hodgkin lymphoma), acute lymphoblastic and myeloblastic leukemia, childhood neuroblastoma, ovarian cancer, mammary adenocarcinoma. Phelinun in combination with other cytotoxic medicinal products is indicated as reduced intensity conditioning (RIC) treatment prior to allogeneic haematopoietic stem cell transplantation (allo-HSCT) in malignant haematological diseases in adults. Phelinun in combination with other cytotoxic medicinal products is indicated as conditioning regimen prior to allogeneic haematopoietic stem cell transplantation in haematological diseases in the paediatric population as: Myeloablative conditioning (MAC) treatment in case of malignant haematological diseases RIC treatment in case of non-malignant haematological diseases. source ↗
2020emaOriginal application approval — Acalabrutinib· Calquence as monotherapy or in combination with obinutuzumab is indicated for the treatment of adult patients with previously untreated chronic lymphocytic leukaemia (CLL).Calquence in combination with venetoclax with or without obinutuzumab is indicated for the treatment of adult patients with previously untreated chronic lymphocytic leukaemia (CLL).Calquence as monotherapy is indicated for the treatment of adult patients with chronic lymphocytic leukaemia (CLL) who have received at least one prior therapy.Calquence in combination with bendamustine and rituximab (BR) is indicated for the treatment of adult patients with previously untreated mantle cell lymphoma (MCL) who are not eligible for autologous stem cell transplant (ASCT).Calquence as monotherapy is indicated for the treatment of adult patients with relapsed or refractory mantle cell lymphoma (MCL) not previously treated with a BTK inhibitor. source ↗
Other regulatory activity
2026emaProduct authorisation ended — 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 ↗
2025emaProduct authorisation ended — 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 ↗
2024emaProduct authorisation ended — 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.