PCNSL

Disease

Disease Profile

PCNSL stands for primary central nervous system lymphoma, an aggressive extranodal non-Hodgkin lymphoma confined to the brain, spinal cord, leptomeninges, or eyes at diagnosis.

Category: non-Hodgkin lymphoma
Prevalence: Population incidence is approximately 0.4 to 0.5 per 100,000 person-years.

Related News

FDA Accepts Deciphera's NDA for Tirabrutinib in Relapsed/Refractory PCNSL

Jun 15, 2026

The U.S. FDA has accepted for filing a New Drug Application for tirabrutinib under the accelerated approval pathway for relapsed or refractory primary central nervous system lymphoma, setting a PDUFA action date of December 18, 2026. The submission is supported by Phase 2 PROSPECT study results showing a 67% overall response rate and 44% complete response rate. If approved, tirabrutinib would be the first BTK inhibitor commercially available in the U.S. for this indication.

FDA Grants Fast Track Designation to Multiple Therapies for Rare Diseases and Hematologic Malignancies

Mar 11, 2026

The FDA has granted fast track designation to QRX003 for Netherton Syndrome and IBI3003 for relapsed or refractory multiple myeloma, while also accepting regulatory applications for other therapies addressing serious conditions with unmet medical needs.

FDA Accepts Tirabrutinib Application for Relapsed Primary CNS Lymphoma

Feb 24, 2026

The FDA accepted a new drug application for tirabrutinib to treat relapsed or refractory primary central nervous system lymphoma, with a decision expected December 18, 2026. The application is supported by phase 2 trial data showing a 67% overall response rate.

FDA Approves First-Line Acalabrutinib Regimen for CLL/SLL, Expands NSCLC Dosing Options

Feb 21, 2026

The FDA approved acalabrutinib plus venetoclax as the first all-oral, fixed-duration BTK inhibitor regimen for CLL/SLL and cleared monthly dosing for amivantamab in EGFR-mutated NSCLC. Additional regulatory reviews are underway for PCNSL and breast cancer treatments.

FDA Accepts Tirabrutinib NDA for Relapsed/Refractory PCNSL, Sets December 2026 Action Date

Feb 18, 2026

The FDA has accepted a new drug application for tirabrutinib, a BTK inhibitor, for relapsed/refractory primary central nervous system lymphoma under the accelerated approval pathway. The regulatory agency set a PDUFA target action date of December 18, 2026.

FDA Accepts Tirabrutinib NDA for Relapsed/Refractory PCNSL with December 2026 PDUFA Date

Feb 16, 2026

The FDA has accepted for filing the New Drug Application for tirabrutinib under the accelerated approval pathway for relapsed or refractory primary central nervous system lymphoma, setting a PDUFA action date of December 18, 2026.

CAR T-Cell Therapy Advances Target Solid Tumors and Selective Cancer Killing

Feb 14, 2026

Researchers have developed modified CAR T cells that overcome prostaglandin E2 suppression in solid tumors and a selective therapy targeting IGHV4-34 that spares healthy immune cells, addressing key limitations in current treatments.

Related Clinical Trials

NCT ID	Title	Status	Phase
NCT07350850	A Multicenter Two-Cohort Study of Methotrexate, Rituximab, Sintilimab and Pirtobrutinib for Treatment-Naive PCNSL vs. Real-World Investigator-Selected Treatment (Observational Cohort)	RECRUITING	PHASE2
NCT06914700	A Nomogram for Predicting the Diagnosis of Central Malignant Tumors Based on Preoperative Clinical Characteristics and Laboratory Indicators:	COMPLETED
NCT06554561	A Study of Pomadomide in Combination With Rituximab and Methotrexate for Newly-diagnosed Primary Central Nervous System Lymphoma	RECRUITING	PHASE1/PHASE2
NCT05896007	Zanubrutinib Combined With Rituximab and Chemotherapy for Newly-Diagnosed PCNSLBCL Patients Intolerant to HSCT	RECRUITING	PHASE2
NCT05782374	Real World Data on Ibrutinib Use in PCNSL Rel/Ref	ACTIVE_NOT_RECRUITING
NCT05167630	Prognosis Factors in Patients With Primary Central Nervous System Lymphoma	UNKNOWN
NCT05021770	Orelabrutinib in Combination With Thiotepa in Refractory and Relapsed Primary CNS Lymphoma	UNKNOWN	PHASE1/PHASE2
NCT04514393	Ibrutinib With Methotrexate and Temozolomide for Patients With Newly Diagnosed Primary CNS Lymphoma	UNKNOWN	PHASE2
NCT04481815	Rituximab Plus Methotrexate With or Without Lenalidomide in Treating Patients With Newly Diagnosed Primary Central Nervous System Lymphoma	RECRUITING	PHASE2
NCT04438044	A Study to Evaluate ICP-022 in Patients With R/R PCNSL and SCNSL	UNKNOWN	PHASE2