Primary brain lymphoma
Primary Brain Lymphoma Primary Brain Lymphoma (PBL) is a rare type of cancer that originates in the lymphatic cells within the brain. It is a form of extranodal non-Hodgkin lymphoma, meaning it occurs outside the lymph nodes. This condition is also known as Primary Central Nervous System Lymphoma (PCNSL).
Pathophysiology[edit | edit source]
PBL is characterized by the proliferation of malignant lymphocytes, typically B-cells, within the central nervous system (CNS). The exact cause of PBL is not well understood, but it is associated with immunosuppression, such as in patients with HIV/AIDS or those who have undergone organ transplantation.
Symptoms[edit | edit source]
The symptoms of PBL can vary depending on the location and size of the tumor. Common symptoms include:
- Headaches
- Seizures
- Cognitive or personality changes
- Focal neurological deficits, such as weakness or numbness
Diagnosis[edit | edit source]
Diagnosis of PBL involves a combination of imaging studies and biopsy. Magnetic Resonance Imaging (MRI) is typically used to identify lesions in the brain. A definitive diagnosis is made through a biopsy, where a sample of the tumor is examined histologically.
Treatment[edit | edit source]
Treatment for PBL often involves a combination of therapies:
- Chemotherapy: High-dose methotrexate is commonly used.
- Radiation therapy: Whole-brain radiation therapy may be employed, although it is associated with significant neurotoxicity.
- Corticosteroids: These are used to reduce inflammation and edema around the tumor.
Prognosis[edit | edit source]
The prognosis for PBL varies. Factors influencing prognosis include the patient's age, performance status, and response to initial treatment. The median survival time has improved with advances in treatment, but long-term survival remains challenging.
Epidemiology[edit | edit source]
PBL is a rare disease, accounting for approximately 1% of all brain tumors and 4% of all extranodal lymphomas. It is more common in immunocompromised individuals.
Research and Future Directions[edit | edit source]
Research is ongoing to better understand the pathogenesis of PBL and to develop more effective treatments. Clinical trials are exploring new chemotherapy regimens and targeted therapies.
See Also[edit | edit source]
External Links[edit | edit source]
- [National Cancer Institute - Primary CNS Lymphoma](https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq)
- [American Brain Tumor Association - Primary CNS Lymphoma](https://www.abta.org/tumor_types/primary-central-nervous-system-lymphoma/)
NIH genetic and rare disease info[edit source]
Primary brain lymphoma is a rare disease.
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Contributors: Prab R. Tumpati, MD
