Overview
Primary central nervous system lymphoma (PCNSL) is a rare and aggressive form of extranodal non-Hodgkin lymphoma primarily affecting the brain, spinal cord, cerebrospinal fluid, and eyes 1.Diagnosis
Biopsy: Essential for definitive diagnosis 1.
Imaging: MRI is crucial for tumor localization and staging 1.
Cerebrospinal fluid analysis: Often demonstrates malignant cells 1.Management
First-line treatment: Chemotherapy, typically high-dose methotrexate-based regimens 1.
Adjunctive treatments:
- CAR T-cell therapy: Shows promise in relapsed/refractory cases with notable toxicity (CRS, ICANS) 2.
- Surgical resection: Limited to biopsy; debated role in selected cases with superficial, single-lesion tumors 3.Special Populations
Resource-limited settings: Emphasize early diagnosis and tailored treatment approaches due to resource constraints 1.Key Recommendations
Early diagnosis and prompt initiation of chemotherapy are critical for improving outcomes in PCNSL (Evidence: Strong 1).
Consider surgical resection for diagnostic purposes only in cases with superficial, single-lesion tumors, though overall survival benefits are not definitively established (Evidence: Moderate 3).
Evaluate CAR T-cell therapy for relapsed/refractory PCNSL patients, acknowledging significant associated toxicities (Evidence: Weak 2).References
1 Aziz HF, Bakhshi SK, Ahmed N, Bajwa MH, Jabbar AA, Hafiz A et al.. Consensus guidelines for the management of primary central nervous system lymphoma for low and middle-income countries. JPMA. The Journal of the Pakistan Medical Association 2024. link
2 Cook MR, Dorris CS, Makambi KH, Luo Y, Munshi PN, Donato M et al.. Toxicity and efficacy of CAR T-cell therapy in primary and secondary CNS lymphoma: a meta-analysis of 128 patients. Blood advances 2023. link
3 Stifano V, Della Pepa GM, Offi M, Montano N, Carcagnì A, Pallini R et al.. Resection versus biopsy for management of primary central nervous system lymphoma: a meta-analysis. Neurosurgical review 2023. link