Primary CNS lymphoma
Primary CNS Lymphoma[edit | edit source]
Primary CNS Lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma that is confined to the central nervous system (CNS), including the brain, spinal cord, leptomeninges, and eyes. It is a type of lymphoma that originates in the lymphatic system but is unique in its localization to the CNS.
Epidemiology[edit | edit source]
PCNSL accounts for approximately 1-2% of all brain tumors and 4-6% of extranodal lymphomas. It is more common in immunocompromised individuals, such as those with HIV/AIDS or organ transplant recipients, but can also occur in immunocompetent individuals. The incidence of PCNSL has increased over the past few decades, particularly in the context of the HIV epidemic.
Pathophysiology[edit | edit source]
PCNSL is typically a diffuse large B-cell lymphoma (DLBCL), which is a subtype of non-Hodgkin lymphoma. The exact pathogenesis is not fully understood, but it is believed to involve the transformation of B-cells within the CNS. The blood-brain barrier plays a significant role in the disease's unique presentation and challenges in treatment.
Clinical Presentation[edit | edit source]
Patients with PCNSL may present with a variety of neurological symptoms depending on the location of the tumor. Common symptoms include:
- Headaches
- Seizures
- Cognitive or personality changes
- Focal neurological deficits
- Visual disturbances (if the eyes are involved)
Diagnosis[edit | edit source]
The diagnosis of PCNSL involves a combination of imaging studies, such as MRI, and histopathological examination. A brain biopsy is often required to confirm the diagnosis. Cerebrospinal fluid (CSF) analysis and ocular examination may also be performed if leptomeningeal or ocular involvement is suspected.
Treatment[edit | edit source]
The treatment of PCNSL typically involves high-dose methotrexate-based chemotherapy, often in combination with other agents. Radiation therapy may be used, but its role has diminished due to the risk of neurotoxicity, especially in older patients. Autologous stem cell transplantation is considered in some cases.
Prognosis[edit | edit source]
The prognosis of PCNSL varies depending on several factors, including the patient's age, performance status, and response to treatment. The median survival has improved with modern therapies, but long-term survival remains challenging.
Research and Future Directions[edit | edit source]
Ongoing research is focused on understanding the molecular biology of PCNSL, improving diagnostic techniques, and developing targeted therapies. Clinical trials are exploring new treatment regimens and the role of immunotherapy in PCNSL.
See Also[edit | edit source]
NIH genetic and rare disease info[edit source]
Primary CNS lymphoma is a rare disease.
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Contributors: Prab R. Tumpati, MD
