> Trialight public data. Index of pages: https://trialight.com/llms.txt
> Web page: https://trialight.com/data/condition/primary-central-nervous-system-lymphoma-0f3f2051-d8a7-5ab2-bc09-6bacfa58c99a
> Record changed: 2026-10-13

# Primary central nervous system lymphoma

Therapeutic area

Immunology

Rare disease

Yes

## Definition and classification

**Definition**

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.

**Genetic basis**

No

**Non-human condition**

No

**Rare disease**

Yes

**Listing at source**

Listed

**Condition name**

Primary central nervous system lymphoma

**Therapeutic area**

Immunology

### Alternative names (12)

- primary central nervous system lymphoma
- 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 central nervous system lymphoma
- primary lymphoma, CNS

## Clinical profile

**Disease mechanisms**

- Acquired

**Severity**

Severe

### All therapeutic areas (4)

- Hematology
- Immunology
- Neurology
- Oncology

## Identifiers

**MONDO ID**

MONDO:0002571

Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0002571)

**Parent condition**

MONDO:0000621

## Related records

### Broader conditions (33)

- [B-cell neoplasm](https://trialight.com/data/condition/b-cell-neoplasm-ffd0710d-7dbd-55bc-a4a5-3c1cf532bac2.md)
- [Cancer](https://trialight.com/data/condition/cancer-703b1dfc-4812-50eb-83f4-d7a205d16bdb.md)
- Cancer or benign tumor
- Central nervous system cancer
- [Central nervous system disorder](https://trialight.com/data/condition/central-nervous-system-disorder-78230ae5-4e6b-5495-ac04-050b0e11e964.md)
- Central nervous system hematopoietic neoplasm
- [Central nervous system neoplasm](https://trialight.com/data/condition/central-nervous-system-neoplasm-bd35c306-06e5-549c-a882-45bdfbcbb7d8.md)
- Disease
- Disease by body system or component
- Disease by etiologic mechanism
- Disease of primarily extrinsic mechanism
- Epstein-Barr virus-associated lymphoproliferative disorder
- Epstein-Barr virus-related tumor
- Hematologic disorder
- [Hematopoietic and lymphoid cell neoplasm](https://trialight.com/data/condition/hematopoietic-and-lymphoid-cell-neoplasm-ed3616fa-22fe-5984-94b4-745416e01ef9.md)
- [Hematopoietic and lymphoid system neoplasm](https://trialight.com/data/condition/hematopoietic-and-lymphoid-system-neoplasm-510101ac-7ce5-5dd1-8980-f2f110effdf0.md)
- Human disease
- Immune system cancer
- [Immune system disorder](https://trialight.com/data/condition/immune-system-disorder-24846188-b462-515c-bb34-142255a648c8.md)
- Leukocyte disorder

### Narrower conditions (5)

- Central nervous system anaplastic large cell lymphoma
- Central nervous system non-hodgkin lymphoma
- Cerebral lymphoma
- Diffuse large B-cell lymphoma of the central nervous system
- Spinal cord lymphoma

### Trials (193)

- \[18F\]-Fludarabine PET/MRI in Primary Central Nervous System Lymphoma
- \[68Ga\]Ga-PentixaFor PET Imaging in CNS Lymphoma Patients
- \[90Y\]Y-PTT Endoradiotherapy in CNS Lymphoma Patients
- \[CREMA\]Combination of R-M Followed by R-A in Elderly Patients With Primary CNS Lymphoma
- 19(T2)28z1xx Chimeric Antigen Receptor (CAR) T Cells in People With B-Cell Cancers
- A Case Study of Nivolumab for Relapsed/Refractory Primary Central Nervous System Lymphoma
- A Clinical Study Evaluating the Safety and Efficacy of ZRMT Regimen in the Treatment of PCNSL
- A Dose-escalating Pilot Study of Orelabrutinib for Newly-diagnosed PCNSL
- A Multicenter, Prospective Clinical Trial With a Concurrent Control Evaluating Methotrexate Combined With Rituximab,Sintilimab and Pirtobrutinib vs. Investigator-Selected Standard of Care in Treatment-Naive PCNSL
- A Nomogram for Predicting the Diagnosis of Central Malignant Tumors Based on Preoperative Clinical Characteristics and Laboratory Indicators:
- 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
- A Phase I/II Study to Investigate the Use of VORAXAZE™ As Intended Intervention in Patients with CNSL
- a Phase II Study in Primary Central Nervous System Lymphoma
- A Phase II Trail of Chidamide ,Rituximab and Methotrexate in Lymphoma Patients
- A Pilot Study of Acalabrutinib in Relapsed/Refractory Primary and Secondary CNS Lymphomas
- A Pilot Study of Inpatient Hospice With Procurement of Brain Tumor Tissue on Expiration for Research Purposes
- A Prospective Clinical Study of BTK Inhibitor, PD-1 and Formustine in the First-line Treatment of Primary Central Nervous System Lymphoma
- A Safety Study of Rituximab Plus MTX Injected Into the Cerebrospinal Fluid in the Treatment of Brain Lymphoma
- A Study of Epcoritamab and Ibrutinib in People With Central Nervous System Lymphoma (CNSL)
- A Study of F520 in Relapsed/Refractory Primary Central Nervous System Lymphoma (PCNSL) or Secondary Central Nervous System Lymphoma (SCNSL)

### Drugs in development (25)

- Acalabrutinib Phase 2
- Bimiralisib Phase 2
- Camrelizumab Phase 2
- Cytarabine Phase 2
- [Dexamethasone](https://trialight.com/data/asset/dexamethasone-b923ddae-f095-408c-96e9-975238a429ad.md) Phase 1
- [Filgrastim](https://trialight.com/data/asset/filgrastim-942bf0d3-0bf1-4bf8-9e38-81a6c277d728.md) Phase 2
- [Ibrutinib](https://trialight.com/data/asset/ibrutinib-0b3ac4fd-6e98-4847-a711-2f599e8bb93d.md) Phase 2
- Idarubicin Phase 2
- [Lenalidomide](https://trialight.com/data/asset/lenalidomide-1e614dfb-efd0-4006-a121-30a97656c7a9.md) Phase 2
- Lisocabtagene maraleucel Phase 2
- Loncastuximab Phase 1
- [Methotrexate](https://trialight.com/data/asset/methotrexate-aa7ef9da-5b6e-44a1-942c-610e3de14a1d.md) Phase 3
- [Nivolumab](https://trialight.com/data/asset/nivolumab-d2e207ca-a7d1-43d0-987f-43ef7eef8f1b.md) Phase 2
- [Obinutuzumab](https://trialight.com/data/asset/obinutuzumab-1aa4994f-1407-4a89-93f0-0b3aa295ad6e.md) Phase 2
- [Orelabrutinib](https://trialight.com/data/asset/orelabrutinib-f8934fae-2fe2-4ce8-a944-a872e27bfa47.md) Phase 3
- [Pembrolizumab](https://trialight.com/data/asset/pembrolizumab-f9ac1934-47a5-4a53-ae55-20b7d53d8815.md) Phase 2
- [Pemetrexed](https://trialight.com/data/asset/pemetrexed-2221859e-676b-4d76-97df-e11f3f9664a1.md) Phase 2
- Penpulimab Phase 2
- [Rituximab](https://trialight.com/data/asset/rituximab-3fb73477-f618-414e-aa0a-9ccba4d6da3f.md) Phase 3
- [Temozolomide](https://trialight.com/data/asset/temozolomide-3fac1b09-bcc5-4875-871e-2cc6460dc731.md) Phase 3

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