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

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

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

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

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

The treatment of PCNSL typically involves chemotherapy, often in combination with radiation therapy. The most commonly used chemotherapy regimen is high-dose methotrexate.

Prognosis

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

• Lymphoma
• Non-Hodgkin lymphoma
• Brain tumor

PCNSL Resources
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