Central Nervous System Lymphoma: Classification, Symptoms, Diagnosis, and Treatment Approaches

What Are Central Nervous System (CNS) Lymphoma? 

Central Nervous System (CNS) lymphoma is an uncommon but highly aggressive cancer affecting the brain or spinal cord. Unlike cancers that spread to the brain from other parts of the body, primary central nervous system lymphoma cancer develops directly in the brain or spinal cord. This condition is commonly seen in individuals with weakened immune systems, such as those with HIV/AIDS or organ transplant recipients, but it can also occur in immunocompetent individuals. CNS lymphoma is generally classified as one of the types of non-Hodgkin lymphoma (NHL), and if not diagnosed and treated promptly, it can lead to severe consequences.

Types and Classification of CNS Lymphoma

CNS lymphoma can be categorized into different types based on its origin and response to treatment. The main categories are:

1. Primary CNS Lymphoma (PCNSL)

This type of lymphoma originates directly in the brain, spinal cord, or eye, with no other cancer present in the body. It is typically classified as a B-cell lymphoma and often appears in the cerebral hemispheres, but it can also affect the spinal cord and eyes (ocular lymphoma).

2. Secondary CNS Lymphoma

Secondary CNS lymphoma develops when lymphoma from another part of the body spreads to the brain or spinal cord. It is rarer than primary CNS lymphoma but can occur in individuals who already have a diagnosis of lymphoma elsewhere in the body.

Common Symptoms of CNS Lymphoma

The symptoms of CNS lymphoma vary depending on the tumor’s location and size. Common symptoms include:

Headaches: Persistent and progressively worsening headaches that do not respond to conventional painkillers.

Neurological Disorders: Weakness in muscles, numbness, and loss of coordination may result from the tumor affecting motor pathways.

Seizures: Disruption of electrical activity in the brain can lead to seizures.

Diagnostic Methods

The diagnosis of CNS lymphoma is made through clinical evaluation, imaging studies, and tissue biopsy:

Neurological Examination: Evaluates reflexes, muscle strength, coordination, and cognitive functions.

Magnetic Resonance Imaging (MRI): In primary central nervous system lymphoma MRI: The most commonly used imaging method to detect brain tumors. MRI reveals the size and spread of the tumor.

Positron Emission Tomography (PET) Scan: Imaging helps identify active lymphoma cells and determines if the tumor has extended to the brain or other parts of the body.

Stereotactic Biopsy: Biopsy, either through surgical intervention or stereotactic needle biopsy, is necessary to confirm the diagnosis and determine if the lymphoma is B-cell or T-cell type.

Treatment of CNS Lymphoma

Treatment of CNS lymphoma requires a multidisciplinary approach.

1. Chemotherapy

Is essential in treating CNS lymphoma. Due to the aggressive nature and diffuse spread of lymphoma cells in the brain and spinal cord, chemotherapy plays a critical role in controlling the disease and improving patient outcomes.

2. Radiotherapy

Radiotherapy is used in conjunction with chemotherapy and is particularly applied when chemotherapy alone is insufficient. Whole-brain radiotherapy (WBRT) is frequently used in brain tumors but may be associated with cognitive side effects, so it is used cautiously.

3. Targeted Therapy and Immunotherapy

Targeted Therapy: Drugs targeting genetic mutations in lymphoma cells are used. Immunotherapy: An innovative approach that helps the immune system fight lymphoma.