Primary CNS Lymphoma

About Primary CNS Lymphoma

General Information

• Primary central nervous system (CNS) lymphoma is a malignant tumor derived from white blood cells that is localized to the nervous system.
• These tumors represent 0.85 percent to 2 percent of all primary brain tumors; however, the incidence is rising relative to other brain lesions. The increase is due in part to the occurrence of primary lymphoma in AIDS and transplant patients.
• The median age of diagnosis is 52, or approximately 34 in immunocompromised patients.
• Most common locations are the frontal lobes, then deep nuclei and periventricular zone.
• Conditions with increased risk of primary CNS lymphomas:
  • Collagen vascular disease.
    • Systemic lupus erythematosus.
    • Sjogren's syndrome.
    • Rheumatoid arthritis.
  • Immunosuppression.
    • Chronic immunosuppression in transplant patients.
    • Severe congenital immunodeficiency syndrome.
    • CNS lymphoma occurs in 19 percent of AIDS patients.
  • Epstein-Barr virus.

Symptoms

• Non-focal, non-specific symptoms occur in more than 50 percent of patients; mental status changes in one third; symptoms of increased intracranial pressure (headache, nausea/vomiting) and/or generalized seizures in 9 percent.
• Focal symptoms in 30 percent to 42 percent of cases, including weakness or numbness, partial seizures and cranial nerve palsies (visual changes, double vision, facial numbness, facial weakness, hearing loss and/or swallowing difficulties).

Diagnosis

• Lymphomas are primarily diagnosed with computed tomography (CT) or magnetic resonance imaging (MRI) brain scans. On imaging, 50 percent to 60 percent occur in multiple areas.
• All patients should be assessed (history, physical and laboratory tests) for conditions associated with lymphoma. Since primary CNS lymphoma is very rare, any patient with CNS lymphoma should have a work-up for systemic lymphoma:
  • Careful physical examination of all lymph nodes.
  • Chest x-ray and CT scan of chest and abdomen.
  • Routine blood and urine testing.
  • Bone marrow biopsy.
  • Testicular ultrasound in males.
  • Eye exam.

Treatment

• Surgery: Surgical decompression with partial or total removal does not alter patient's prognosis.
  • The main role of surgery is for tumor biopsy
  • Stereotactic techniques are often well suited for these frequently deep tumors.
• Steroid Therapy: These tumors are steroid sensitive.
  • Within hours the brain lesions may be reduced or may disappear as tumor cells are killed.
  • The benefits of steroids tend to disappear within six months of treatment but may last longer.
• Radiation Therapy: These tumors respond quickly and effectively to external radiation.
  • Complete responses have been reported in 80 percent of patients. However, recurrence within the radiation field is the rule.
  • The optimal volume to be irradiated depends on the nature of the tumor and the tolerance of the critical normal tissue.
• Chemotherapy: Because of the generally poor long-term results with radiation only, and the high rate of local failure, considerable attention has been focused recently on the role of systemic chemotherapy. Several reports have suggested improved survival when chemotherapy is added to radiation.

The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. We work in close cooperation with your surgeon or medical doctor with whom you have had initial contact. Together with the surgeon or medical doctor, the Neuro-ICU attending physician and team members direct your family member's care while in the ICU. The Neuro-ICU team consists of the bedside nurses, nurse practitioners, physicians in specialty training (Fellows) and attending physicians. UCLA Neuro ICU Family Guide