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Primary Central Nervous System CNS Lymphoma

GENERAL QUESTIONS 

o How common is a primary central nervous system lymphoma? 
o Does the location of the lymphoma make a difference? 

DIAGNOSIS

o What are symptoms of a lymphoma in the brain? 
o What tests are needed for the initial evaluation? 
 

TREATMENTS 

o A team of doctors to treat my lymphoma 
o Neurosurgery 
o Radiation Therapy .. How the role of radiation therapy has changed 
o Chemotherapy and other drugs .. Why chemotherapy is given before radiation therapy 
o Immunotherapy – How is it used? 
o Steroids – How are they used? 
o My chances for longer life or cure with a lymphoma

GENERAL QUESTIONS 

How common are primary central nervous system 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 lymphoma make a difference? 

Yes. Location can affect the diagnostic tests and therapy.

• Lymphoma originating in the brain are still unusual, so it’s imperative that a search begin in all lymph node areas (neck, groin, chest, abdomen), since lymphomas usually start there.
• Tumors in the spine will require chemotherapy into the spinal canal, chemotherapy into a reservoir in the brain, or radiation to the spine. 

DIAGNOSIS

What are symptoms of a lymphoma in the brain? 

Typical symptoms reflect the area affected by the tumor. Most 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 lymphoma? 

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

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

TREATMENT 

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

Remember: 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. Below is a case example of what might happen when a team is not involved: 

Gerald is a 49-year-old southern Californian executive who two years earlier had a mild stroke. He was treated with steroids and then rehabilitation. Gerald was told that he had a “brain tumor” that caused arm weakness. His local oncologist told him that the tumor could be either a glioma or “secondary” lymphoma (originating from elsewhere). Their team said that a biopsy was unnecessary, as he would need radiation therapy anyway. In the meantime, Gerald had received a week of steroids to lessen the swelling in the brain and return function to his arm. Gerald came to see me for a second opinion. We took an MRI scan and the tumor was gone! This disappearance after steroids is almost proof of a lymphoma. We waited eight weeks, took another scan, and sure enough the tumor had inched back near his brain stem (pons), next to the spinal cord. Dr. Keith Black performed careful biopsies around the brain stem area and on the fourth “pinch” sample of tumor; a B-cell-type lymphoma was diagnosed. Gerald received high dose intravenous methotrexate and Rituxan antibody therapy, followed by radiation. He is well four years later.

Without the biopsy, however, Gerald would have received only radiation, which was not the correct therapy. 

Surgery 

Neurosurgery for a 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 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 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 into one 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

What medications will I receive before or during surgery? 

See Chapter 9, Medications. In the book 

Radiation Therapy 

How has the role of radiation therapy changed in the treatment of 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 to months.^{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 lymphomas? 

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

Specific radiation techniques used on brain tumors are:

• Radiosurgery
  Radiosurgery is not really surgery because no incision is required. Focused radiation is used to destroy a tumor. Because the radiation is focused, a smaller dose can be used. Several machines, including a gamma knife and a linear accelerator, can produce this type of radiation. When a gamma knife is used, an imaging frame is attached to the person's skull. The person lies on a sliding bed, and a large helmet with holes in it is placed over the frame. The head of the bed is then slid into a globe that contains radioactive cobalt. Radiation passes through the holes in the helmet and is aimed precisely at the tumor. A linear accelerator circles the head of the person, who lies on a sliding bed. The linear accelerator aims radiation precisely at the tumor. ⁴

Chemotherapy and other drugs 

Why is chemotherapy given before radiation therapy for 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 (Table 14-1 in the book). 

Dr. Lisa DeAngelis in New York has described a fairly standard “chemotherapy first” approach.³ (See Brain Tumors- Finding the Ark for more information in the book, Chapter 19: Medications, section on regional chemotherapy).

Immunotherapy – How is it used to treat lymphomas? 

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. Several new therapeutic monoclonal antibodies target CD-20 including Rituxan and Bexxar.

Steroids – How are they used in treatment of lymphomas? 

Dexamethasone (Decadron) is the strongest steroid drug in clinical practice, and it is frequently administered to brain tumor patients to reduce swelling and “tightness.” It has a unique role in diagnosis and therapy of lymphomas. (See Chapter 9 in the book for more information) 

What are my chances of remission with a lymphoma? 

Remission depends upon many factors, some of the most important include: 

• Accurate diagnosis of lymphoma 
• Accurate subtyping of lymphoma, B or T cell? 
• AIDS-associated or not.
• Location: Lymphoma only in the central nervous system or metastasis
   from elsewhere? The other sites require surgery or radiation fields. 
• Development while on or off chemotherapy. 
• Receipt of correct therapy (e.g. the correct sequence of treatments) will
   determine outcome and toxicity (radiation and chemotherapy). 
• Response to the previous and current therapies 

See Brain Tumors: Leaving the Garden of Eden for more information.

Footnotes

¹ Nelson DF, Martz KL, Bonner H. et al Non-Hodgkin's lymphoma of the brain: can high dose, large volume radiation therapy improve survival? Report on a prospective trial by the Radiation Therapy Oncology Group (RTOG): RTOG 8315.. Int J Radiat Oncol Biol Phys. 1992;23(1):247-8.

² Leibel SA, Sheline GE Radiation therapy for neoplasms of the brain.. J Neurosurg. 1987 Jan;66(1):1-22. 

³ Abrey LE, Yahalom J, DeAngelis L Treatment for primary CNS lymphoma: the next step.. J Clin Oncol. 2000 Sep;18 (17):3144-50. 

⁴ Merck Manual - Brain Tumors 2/1/03 (near bottom of page)

Adult Non-Hodgkin's Lymphoma Information Pages:

• Adult Non-Hodgkin's Lymphomas
• Lymphoma: Classification and Typing
• Non-Hodgkin's Lymphoma: Aggressive Lymphomas
• Non-Hodgkin's Lymphoma: Indolent Lymphomas
• Adult Non-Hodgkin's Lymphoma: Diagnosis
• Adult Non-Hodgkin's Lymphoma: Treatment

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• Childhood Lymphoma
• Childhood Non-Hodgkin's Lymphoma
• Childhood Non-Hodgkin's Lymphoma: Diagnosis
• Childhood Non-Hodgkin's Lymphoma: Types and Staging
• Childhood Non-Hodgkin's Lymphoma: Treatment
• Childhood Lymphoma: Resources

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Compilation Copyright © 1998-2005 Lymphoma Information Network http://www.LymphomaInfo.net/ - All Rights Reserved.  

Portions 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.

Updated November 20, 2005