PCNSL
PCNSL or Primary Central Nervous System Lymphoma is a type of lymphoma, a cancer that originates from the lymphocytes, which is restricted to the brain, eyes, leptomeninges, or spinal cord. It is a rare form of Non-Hodgkin lymphoma that is aggressive and fast-growing.
Epidemiology[edit | edit source]
PCNSL accounts for approximately 3% of all primary brain tumors and 4% of all extranodal lymphomas. The incidence of PCNSL has been increasing over the past few decades, particularly in individuals over the age of 60.
Pathophysiology[edit | edit source]
The exact cause of PCNSL is unknown. However, it is known that the disease arises from B cells, a type of white blood cell. These cells undergo malignant transformation and proliferate in an uncontrolled manner, leading to the formation of a tumor.
Clinical Presentation[edit | edit source]
Patients with PCNSL typically present with neurological symptoms, which can vary depending on the location of the tumor. Common symptoms include headache, seizures, cognitive impairment, and focal neurological deficits.
Diagnosis[edit | edit source]
The diagnosis of PCNSL is typically made based on imaging studies, such as MRI or CT scan, and confirmed with a biopsy of the tumor. The biopsy can show the presence of atypical lymphoid cells, which is indicative of lymphoma.
Treatment[edit | edit source]
The treatment of PCNSL typically involves chemotherapy, often in combination with radiation therapy. The most commonly used chemotherapy regimen is high-dose methotrexate.
Prognosis[edit | edit source]
The prognosis of PCNSL is generally poor, with a median survival of approximately one year. However, survival can be significantly improved with aggressive treatment.
See Also[edit | edit source]
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Contributors: Prab R. Tumpati, MD