Disease · MONDO_0002571

primary central nervous system lymphoma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. primary central nervous system lymphoma. Diseases, record MONDO_0002571. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0002571

On this page

Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: primary central nervous system lymphoma; Open Targets
Open Targets ID: MONDO_0002571; Open Targets
Other names: 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; Open Targets
Disease Ontology: DOID:3234; Disease Ontology
NCI Thesaurus: C9301; NCI Thesaurus
Orphanet: ORPHA:46135; Orphanet
Highest stage of its drugs: Phase 3, for 14 of its 97 drugs, from ClinicalTrials.gov; Open Targets

Drugs97

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
METHOTREXATE	CHEMBL34259	Small molecule	Phase 3	ClinicalTrials.gov	74	74	74	Drugs and targets
RITUXIMAB	CHEMBL1201576	Antibody	Phase 3	ClinicalTrials.gov	71	71	71	Drugs and targets
CYTARABINE	CHEMBL803	Small molecule	Phase 3	ClinicalTrials.gov	27	27	27	Drugs and targets
TEMOZOLOMIDE	CHEMBL810	Small molecule	Phase 3	ClinicalTrials.gov	26	26	26	Drugs and targets
THIOTEPA	CHEMBL671	Small molecule	Phase 3	ClinicalTrials.gov	18	18	18	Drugs and targets
ORELABRUTINIB	CHEMBL4650321	Small molecule	Phase 3	ClinicalTrials.gov	16	16	16	Drugs and targets
DEXAMETHASONE	CHEMBL384467	Small molecule	Phase 3	ClinicalTrials.gov	13	13	13	Drugs and targets
PROCARBAZINE	CHEMBL1321	Small molecule	Phase 3	ClinicalTrials.gov	13	13	13	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 3	ClinicalTrials.gov	11	11	11	Drugs and targets
CARMUSTINE	CHEMBL513	Small molecule	Phase 3	ClinicalTrials.gov	7	7	7	Drugs and targets

1 to 10 of 97

See all 97 drugs

Targets74

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
BTK	Tyrosine-protein kinase BTK	7	Tyrosine-protein kinase BTK inhibitor	Phase 3	https://conductscience.org/library/genes/695	Drugs and targets
PDCD1	Programmed cell death protein 1	6	Programmed cell death protein 1 antagonist; Programmed cell death protein 1 inhibitor	Phase 2	https://conductscience.org/library/genes/5133	Drugs and targets
TOP2A	DNA topoisomerase 2-alpha	6	DNA topoisomerase II alpha inhibitor; DNA topoisomerase II inhibitor	Phase 3	https://conductscience.org/library/genes/7153	Drugs and targets
CD19	B-lymphocyte antigen CD19	5	B-lymphocyte antigen CD19 binding agent	Phase 2	https://conductscience.org/library/genes/930	Drugs and targets
MS4A1	B-lymphocyte antigen CD20	4	B-lymphocyte antigen CD20 binding agent	Phase 3	https://conductscience.org/library/genes/931	Drugs and targets
DHFR	Dihydrofolate reductase	3	Dihydrofolate reductase inhibitor	Phase 3	https://conductscience.org/library/genes/1719	Drugs and targets
NR3C1	Glucocorticoid receptor	3	Glucocorticoid receptor agonist	Phase 3	https://conductscience.org/library/genes/2908	Drugs and targets
PIK3CA	PI3-kinase class I	3	PI3-kinase class I inhibitor; PI3-kinase p110-alpha subunit inhibitor	Phase 2	https://conductscience.org/library/genes/5290	Drugs and targets
PIK3CD	PI3-kinase class I	3	PI3-kinase class I inhibitor; PI3-kinase p110-delta subunit inhibitor	Phase 2	https://conductscience.org/library/genes/5293	Drugs and targets
RRM1	Ribonucleoside-diphosphate reductase RR1	3	Ribonucleoside-diphosphate reductase RR1 inhibitor	Phase 2	https://conductscience.org/library/genes/6240	Drugs and targets

1 to 10 of 74

See all 74 targets

Trials176

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT01011920	Trial for Patients With Newly Diagnosed Primary Central Nervous System (CNS) Lymphoma	Phase 2	227	2026-03-19	Central Nervous System Lymphoma	CARMUSTINE, CYTARABINE, METHOTREXATE, RITUXIMAB, THIOTEPA	Its record names the disease	ClinicalTrials.gov
NCT03484702	Trial to Determine the Efficacy and Safety of JCAR017 in Adult Participants With Aggressive B-Cell Non-Hodgkin Lymphoma	Phase 2	113	2024-12-27	Lymphoma, Non-Hodgkin	LISOCABTAGENE MARALEUCEL	Its record names the disease	ClinicalTrials.gov
NCT01399372	Rituximab, Methotrexate, Vincristine Sulfate, Procarbazine Hydrochloride, and Cytarabine With or Without Radiation Therapy in Treating Patients With Primary Central Nervous System Lymphoma	Phase 2	91	2021-05-13	Chemotherapeutic Agent Toxicity, Cognitive/Functional Effects, Lymphoma, Neurotoxicity, Radiation Toxicity	CYTARABINE, METHOTREXATE, PROCARBAZINE, RITUXIMAB, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT02329080	New Combination of Chemoimmunotherapy for Systemic B-cell Lymphoma With Central Nervous System Involvement	Phase 2	79	2025-07-08	Diffuse Large B-cell Lymphoma	CARMUSTINE, CYTARABINE, ETOPOSIDE, FILGRASTIM, FOLINIC ACID, IFOSFAMIDE, METHOTREXATE, RITUXIMAB, THIOTEPA	Its record names the disease	ClinicalTrials.gov
NCT02857426	A Study of Nivolumab in Relapsed/Refractory Primary Central Nervous System Lymphoma (PCNSL) and Relapsed/Refractory Primary Testicular Lymphoma (PTL)	Phase 2	66	2020-10-28	Lymphoma	NIVOLUMAB	Its record names the disease	ClinicalTrials.gov
NCT00068250	Combination Chemotherapy, Monoclonal Antibody, and Radiation Therapy in Treating Patients With Primary Central Nervous System Lymphoma	Phase 1/2	60	2018-02-07	Brain and Central Nervous System Tumors, Lymphoma	METHOTREXATE, RITUXIMAB, TEMOZOLOMIDE	Its record names the disease	ClinicalTrials.gov
NCT00542919	A Study for Patients With Non-Hodgkin's Lymphomas	Phase 2	57	2020-10-19	T-Cell Lymphoma, B-Cell Lymphoma	ENZASTAURIN	Its record names the disease	ClinicalTrials.gov
NCT00594815	Combined Immunochemotherapy Followed By Reduced Dose Radiation Therapy (RT) for Patients With Newly Diagnosed Primary Central Nervous System Lymphoma	Not applicable	52	2017-04-06	Lymphoma	CYTARABINE, LEUCOVORIN, METHOTREXATE, PROCARBAZINE, RITUXIMAB, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT00098774	Rituximab and Combination Chemotherapy in Treating Patients With Newly Diagnosed Primary CNS Lymphoma	Phase 2	47	2014-09-12	Lymphoma	CYTARABINE, ETOPOSIDE, METHOTREXATE, RITUXIMAB, TEMOZOLOMIDE	Its record names the disease	ClinicalTrials.gov
NCT01182415	High Dose Chemotherapy and Stem Cell Transplant for Non-Hodgkin's Lymphoma or Central Nervous System (CNS) Lymphoma	Phase 2	30	2017-12-29	Non-Hodgkin's Lymphoma, CNS Lymphoma	BUSULFAN, CYCLOPHOSPHAMIDE, CYTARABINE, RITUXIMAB, THIOTEPA	Its record names the disease	ClinicalTrials.gov

1 to 10 of 176

See all 176 trials

Comparisons in its trials13

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial	Source
Chemotherapy vs Chemotherapy + Low-Dose WBRT: deaths from any cause	3 of 44	1 of 43	87	NCT01399372	Trial comparisons
Chemotherapy vs Chemotherapy + Low-Dose WBRT: serious adverse events	22 of 43	21 of 43	86	NCT01399372	Trial comparisons
MATRIX - R-ICE - Conditioning and ASCT vs Enrolled, But Not Treated: deaths from any cause	50 of 75	1 of 4	79	NCT02329080	Trial comparisons
MATRIX - R-ICE - Conditioning and ASCT vs Enrolled, But Not Treated: serious adverse events	46 of 75	0 of 4	79	NCT02329080	Trial comparisons
PCNSL Cohort Nivolumab 240 mg (Q2W) vs PTL Cohort Nivolumab 240 mg (Q2W): deaths from any cause	32 of 47	13 of 19	66	NCT02857426	Trial comparisons
PCNSL Cohort Nivolumab 240 mg (Q2W) vs PTL Cohort Nivolumab 240 mg (Q2W): serious adverse events	33 of 47	15 of 19	66	NCT02857426	Trial comparisons
Lenalidomide+Nivolumab Followed by Venetoclax, Ibrutinib,Prednisone, Obinutuzumab, Lenalidomide-Nivo vs Cohort 1, Arm 2: Experimental- VIPOR (Venetoclax, Ibrutinib, Prednisone, Obinutuzumab, Lenalidomide): deaths from any cause	2 of 4	6 of 10	14	NCT05211336	Trial comparisons
Lenalidomide+Nivolumab Followed by Venetoclax, Ibrutinib,Prednisone, Obinutuzumab, Lenalidomide-Nivo vs Cohort 1, Arm 2: Experimental- VIPOR (Venetoclax, Ibrutinib, Prednisone, Obinutuzumab, Lenalidomide): Number of Participants With Serious and/or Non-serious Adverse Events Assessed by the Common Terminology Criteria for Adverse Events (CTCAE v5.0).	4 of 4	9 of 10	14	NCT05211336	Trial comparisons
Lenalidomide+Nivolumab Followed by Venetoclax, Ibrutinib,Prednisone, Obinutuzumab, Lenalidomide-Nivo vs Cohort 1, Arm 2: Experimental- VIPOR (Venetoclax, Ibrutinib, Prednisone, Obinutuzumab, Lenalidomide): serious adverse events	3 of 4	6 of 10	14	NCT05211336	Trial comparisons
Phase I: Temozolomide 100mg vs Phase I: Temozolomide150 mg: Number of Phase I Participants Experiencing Toxicity	1 of 6	3 of 6	12	NCT00068250	Trial comparisons

1 to 10 of 13

See all 13 comparisons in its trials

Papers from its trials91

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
39300447	RESULT	1	PubMed
35834762	RESULT	2	PubMed
34132444	RESULT	1	PubMed
32187361	RESULT	1	PubMed
31187523	RESULT	1	PubMed
31039052	RESULT	1	PubMed
30698644	RESULT	1	PubMed
30620669	RESULT	1	PubMed
30008379	RESULT	1	PubMed
29054815	RESULT	2	PubMed

1 to 10 of 91

See all 91 papers from its trials

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0002571

GET https://conductscience.org/api/diseases/v1/MONDO_0002571/drugs?page=2

API reference For AI assistants