CNS Lymphoma

CNS lymphoma
Micrograph of PCNSL
by Nephron

Parts of this CNS Lymphoma page are an abridged version from CHAPTER 14:– Lymphomas; in Brain Tumors – Leaving the Garden of Eden: A Survival Guide to Learning the Basics, Getting Organized & Finding Your Medical Team. © P.M. Zeltzer (2004). Adapted for the Lymphoma Information Network, 11/01/04, with permission.

Lymphomas in the Brain By Paul M. Zeltzer MD Brain Tumors – Leaving the Garden of Eden: A Survival Guide tO learning the Basics, Getting Organized & Finding Your Medical Team

General Questions

How common are primary CNS lymphomas?

Twenty-five years ago, a PCNSL was a rarity; today they are more common. Why? The increase is due to three groups: a) patients with compromised immune systems who are now living longer (e.g., cancer and AIDS patients), b) patients with lupus, rheumatoid arthritis, and bone marrow transplantation who are receiving immune suppressive therapies; and c) patients who are having (more frequent) biopsies. The latter has led to diagnoses that are more accurate.

Does the location of the CNS lymphoma make a difference?

Yes. Location can affect the diagnostic tests and therapy.

CNS lymphomas originating in the brain are still unusual. It's imperative that a search begin in all lymph node areas (neck, groin, chest, abdomen), since lymphomas usually start there.

Diagnosis

What are symptoms of a CNS lymphoma in the brain?

Typical symptoms reflect the area affected by the tumor. Most CNS lymphomas in the brain grow in the frontal and temporal lobes.

Symptoms include headache, vomiting, forgetfulness, difficulty finding words, confusion, double vision, wobbliness (ataxia), weakness of a leg or arm, and sometimes facial weakness.

What tests are needed for the initial evaluation of a CNS lymphoma?

Most neuron (brain tumor specialists) and other physicians will evaluate a patient with a suspected brain lymphoma in the following manner:

  • Thorough medical history, general physical and neurological examination
  • Brain and spine MRI with and without contrast to visualize the brain and tumor
  • CT scan of the chest, abdominal MRI, or ultrasound including lymph node chains, liver, and spleen (to exclude primary lymphoma elsewhere)
  • Complete blood counts, sedimentation rate, liver and kidney function tests, serum and spinal fluid levels of lactic acid dehydrogenase (LDH)
  • Biopsy (almost always indicated)
  • Analysis of cells in spinal fluid for diagnosis, when a biopsy is dangerous (rare)
  • Evaluation of the tissue specimen or spinal fluid by a Hematopathologist (a pathologist who specializes in diseases of the blood and lymph glands).
  • Special immune marker analysis (immunophenotype) on the lymphoma tissue

Treatment

Why do I need a team of doctors to treat my CNS lymphoma?

Remember: CNS Lymphomas in the brain are uncommon. They require complex management with chemotherapy first, followed by radiation and possibly immune therapy (see below). Evaluation should take place in a comprehensive cancer center where physicians with different areas of expertise can work together. This team might include neurooncologists, neuroradiologists, neurosurgeons, neuropathologists, hematopathologists, neurologists, oncologists, radiation oncologists, endocrinologists, and neuropsychologists. Due to its rarity, families should be encouraged to participate in clinical trials in an attempt to improve and optimize therapy.

Clinical Trials

Cancer Patients have more options through clinical studies. Follow this link to learn more and find a clinical study opportunity near you.

Surgery

Neurosurgery for a CNS lymphoma – Does it help?

The role of neurosurgery for lymphomas is different from other tumors in the brain. For most brain tumors, the more tumor tissue that can be removed (for diagnosis and treatment) by the surgeon, the better the prognosis for longer life. Lymphomas are the exception.

Surgery still remains important for three reasons:

  1. Biopsy to confirm the diagnosis and define the exact type of lymphoma
  2. Preservation or improvement of neurological function
  3. Insertion of a shunt to decrease pressure that cannot be managed by other means.

If my CNS lymphoma is removed or reduced by surgery, are other therapies needed?

Yes! We know that even the best surgeon cannot completely remove all cancer cells; this is even truer for lymphomas. Thus, the after-surgical therapies are critical to your treatment and longer life. I recommend a Tumor Board evaluation or referral to a medical center that specializes in CNS lymphoma treatment.

What is a shunt operation, and why is it necessary?

If a tumor causes pressure within the skull to increase, a shunt may be surgically placed. A shunt is a thin piece of tubing that is inserted intone of the spaces of the brain (ventricles) or sometimes into the space around the spine that contains cerebrospinal fluid (subarachnoid space). The other end of the tubing is threaded under the skin from the head usually to the abdominal cavity. Excess cerebrospinal fluid is drained from the brain and is absorbed in the abdominal cavity. The shunt contains a one-way valve that opens when there is too much fluid in the brain. Shunts may be temporary (until the tumor is removed) or permanent. 4

Radiation Therapy

How has the role of radiation therapy changed in the treatment of CNS lymphoma of the brain?

Twenty years ago, all patients with lymphomas of the brain or spine received immediate radiotherapy and showed dramatic tumor shrinkage within days. The problem was that the tumor returned in weeks tmonths.1, 2 Then, sensitivity of PCNSLs to chemotherapy was not understood.

Now, chemotherapy, usually methotrexate, is the initial treatment at most centers

Irradiation is initiated after chemotherapy has been completed. One exception might be use of emergency irradiation to shrink a spinal tumor that causes paralysis.

What are the different types of radiation therapy for CNS lymphomas?

There are several different techniques used to irradiate lymphomas. See the radiation section for general information on radiation.

Chemotherapy

Why is chemotherapy given before radiation therapy for CNS lymphoma?

Primary central nervous system lymphomas illustrate of how poorly planned therapy can affect survival. There is a reason for the specific sequence of therapies. Radiation to the human brain causes changes in brain cells and blood vessels, which render them exquisitely sensitive to the toxic effects of methotrexate, the best chemotherapy we have for lymphoma.

What is the role of chemotherapy and other drugs for lymphomas?

Chemotherapy has evolved into the major component of successful treatment, paralleling the success of lymphoma therapy in other locations of the body. Fortunately, most lymphomas interrupt the blood-brain-barrier, so drug delivery to the tumor in the brain is not the problem. Several adult and pediatric clinical trials of new chemotherapies and immunotherapies are underway.

Immunotherapy and Radioimmunotherapy

Immunotherapy uses the immune system to recognize, target, and kill tumor cells. Human lymph cells are the best studied and characterized in the body. Scientists know that one protein called CD-20 is on the surface of B-lymphoma cells and not on other normal tissues.

Newer therapeutic monoclonal antibodies target CD-20 including Zevalin, Rituxan and Bexxar.

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