Primary central nervous system lymphoma medical therapy: Difference between revisions
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{{Primary central nervous system lymphoma}} | {{Primary central nervous system lymphoma}} | ||
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==Overview== | ==Overview== | ||
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==Medical Therapy== | ==Medical Therapy== | ||
The treatment of PCNSL depends on the person’s age, performance status and whether or not the person has a decreased ability to fight infections (is immunosuppressed). | |||
Corticosteroids | |||
Corticosteroids | |||
Corticosteroids | |||
Any steroid hormone that acts as an anti-inflammatory by reducing swelling and lowering the body’s immune response (the immune system’s reaction to the presence of foreign substances). are often used to treat PCNSL because it responds very well to corticosteroid therapy. Drugs like prednisone (Deltasone) are used to: | |||
destroy lymphoma cells | |||
relieve swelling (edema) of the brain tissue | |||
When PCNSL is suspected, corticosteroids are usually not given until after diagnostic tests are done because these drugs can significantly decrease the size of tumours and affect test results. | |||
Chemotherapy | |||
Chemotherapy is the main treatment for PCNSL. The chemotherapy drugs commonly used for NHL are not effective against PCNSL because these drugs do not reach the brain or cross the blood-brain barrier. The blood-brain barrier protects the brain and prevents many chemotherapy drugs from reaching brain tumour cells in large enough amounts to destroy them. Chemotherapy drugs used to treat brain tumours because they can cross the blood-brain barrier include: | |||
high-dose methotrexate with leucovorin (folinic acid) rescue | |||
Methotrexate is an important chemotherapy drug for treating PCNSL. | |||
It may be used alone or in combination with other chemotherapy drugs. | |||
It is given into a vein (intravenously). | |||
high dose cytarabine (Cytosar, Ara-C) | |||
PCNSL may also be treated with intrathecal chemotherapy (chemotherapy drugs are injected into the cerebrospinal fluid) or with intraventricular chemotherapy (given through a special small device called an Ommaya reservoir | |||
Ommaya reservoir | |||
A device surgically implanted beneath the scalp that is used to deliver chemotherapy drugs directly into the cerebrospinal fluid (CSF) around the brain and spinal cord.) when lymphoma cells are present in the cerebrospinal fluid (CSF). Whether or not intrathecal or intraventricular chemotherapy is used can also depend on the dose of methotrexate that has been given. | |||
Chemotherapy by itself may be used for elderly people with PCNSL. | |||
Radiation therapy | |||
External beam radiation therapy may also be offered for PCNSL. Radiation therapy is given to the entire brain (whole-brain radiation therapy or WBRT). Radiation therapy may be given on its own or with chemotherapy. When radiation therapy is given with chemotherapy, it is usually given after chemotherapy treatment. The doses of radiation therapy and chemotherapy may need to be adjusted if both treatments are used. | |||
The combination of radiation therapy and chemotherapy can cause severe damage to the nervous system (neurotoxicity), especially in older people with PCNSL. This can result in changes in cognitive functioning, dementia, behaviour changes, balance and coordination problems and other neurologic problems. | |||
Radiation therapy to the eyes is given for people with ocular lymphoma. | |||
Surgery | |||
Surgery does not play a role in the treatment of PCNSL because the tumours are often spread throughout the brain and located deep within the brain. A stereotactic biopsy | |||
stereotactic biopsy | |||
A procedure that uses a 3-dimensional scanning machine ( ultrasound, CT scan or MRI) to find the precise location of a tumour and remove a sample for examination under a microscope. may be done to make a diagnosis. | |||
Recurrent PCNSL | |||
The treatment for recurrent PCNSL depends on the location of the relapse and past treatment. If the person did not receive whole-brain radiation therapy as part of their initial treatment, it may be given for the relapse. Chemotherapy may also be used even if it had been given before, but different drugs may be tried. | |||
Back to top | |||
Treatment in people with immunosuppression | |||
People with AIDS-related PCNSL are treated the same way as people who have a normal immune system, but the treatment is more toxic and can be less effective. AIDS-related PCNSL is often treated with: | |||
anti-HIV drugs, called highly active antiretroviral therapy (HAART) | |||
corticosteroids | |||
whole-brain radiation therapy (WBRT) | |||
Chemotherapy may be given to certain people depending on the status of their immune system. | |||
People who have had organ transplants may need to have their immunosuppressant drug dose decreased or stopped. | |||
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Surgical resection is usually ineffective because of the depth of the tumor. Treatment with irradiation and corticosteroids often only produces a partial response, but tumor recurs in more than 90% of patients. Median survival is 10 to 18 months in immunocompetent patients, and less in those with AIDS. The addition of IV [[methotrexate]] and citrovorum may extend survival to a median of 3.5 years. If radiation is added to methotrexate, median survival may increase beyond 4 years. However, radiation is not recommended in conjunction with methotrexate because of increased risk of leukoencephalopathy and dementia in patients older than 60 years of age<ref> Deangelis LM, Hormigo A. Treatment of primary central nervous system lymphoma. ''Semin Oncol'' 2004; 31:684-692. In AIDS patients, perhaps the most important factor with respect to treatment is the use of highly active anti-retroviral therapy (HAART), which affects the CD4+ lymphocyte population and the level of immunosuppression</ref>. | Surgical resection is usually ineffective because of the depth of the tumor. Treatment with irradiation and corticosteroids often only produces a partial response, but tumor recurs in more than 90% of patients. Median survival is 10 to 18 months in immunocompetent patients, and less in those with AIDS. The addition of IV [[methotrexate]] and citrovorum may extend survival to a median of 3.5 years. If radiation is added to methotrexate, median survival may increase beyond 4 years. However, radiation is not recommended in conjunction with methotrexate because of increased risk of leukoencephalopathy and dementia in patients older than 60 years of age<ref> Deangelis LM, Hormigo A. Treatment of primary central nervous system lymphoma. ''Semin Oncol'' 2004; 31:684-692. In AIDS patients, perhaps the most important factor with respect to treatment is the use of highly active anti-retroviral therapy (HAART), which affects the CD4+ lymphocyte population and the level of immunosuppression</ref>. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Primary CNS lymphoma is a primary intracranial tumor usually present in those with severe immunosuppression --- commonly in those with AIDS --- and represents around 20% of all cases of lymphomas in HIV infection (other types being Burkitt's lymphoma and immunoblastic lymphoma).
Medical Therapy
The treatment of PCNSL depends on the person’s age, performance status and whether or not the person has a decreased ability to fight infections (is immunosuppressed).
Corticosteroids
Corticosteroids Corticosteroids Any steroid hormone that acts as an anti-inflammatory by reducing swelling and lowering the body’s immune response (the immune system’s reaction to the presence of foreign substances). are often used to treat PCNSL because it responds very well to corticosteroid therapy. Drugs like prednisone (Deltasone) are used to:
destroy lymphoma cells relieve swelling (edema) of the brain tissue When PCNSL is suspected, corticosteroids are usually not given until after diagnostic tests are done because these drugs can significantly decrease the size of tumours and affect test results.
Chemotherapy
Chemotherapy is the main treatment for PCNSL. The chemotherapy drugs commonly used for NHL are not effective against PCNSL because these drugs do not reach the brain or cross the blood-brain barrier. The blood-brain barrier protects the brain and prevents many chemotherapy drugs from reaching brain tumour cells in large enough amounts to destroy them. Chemotherapy drugs used to treat brain tumours because they can cross the blood-brain barrier include:
high-dose methotrexate with leucovorin (folinic acid) rescue Methotrexate is an important chemotherapy drug for treating PCNSL. It may be used alone or in combination with other chemotherapy drugs. It is given into a vein (intravenously). high dose cytarabine (Cytosar, Ara-C) PCNSL may also be treated with intrathecal chemotherapy (chemotherapy drugs are injected into the cerebrospinal fluid) or with intraventricular chemotherapy (given through a special small device called an Ommaya reservoir Ommaya reservoir A device surgically implanted beneath the scalp that is used to deliver chemotherapy drugs directly into the cerebrospinal fluid (CSF) around the brain and spinal cord.) when lymphoma cells are present in the cerebrospinal fluid (CSF). Whether or not intrathecal or intraventricular chemotherapy is used can also depend on the dose of methotrexate that has been given.
Chemotherapy by itself may be used for elderly people with PCNSL.
Radiation therapy
External beam radiation therapy may also be offered for PCNSL. Radiation therapy is given to the entire brain (whole-brain radiation therapy or WBRT). Radiation therapy may be given on its own or with chemotherapy. When radiation therapy is given with chemotherapy, it is usually given after chemotherapy treatment. The doses of radiation therapy and chemotherapy may need to be adjusted if both treatments are used.
The combination of radiation therapy and chemotherapy can cause severe damage to the nervous system (neurotoxicity), especially in older people with PCNSL. This can result in changes in cognitive functioning, dementia, behaviour changes, balance and coordination problems and other neurologic problems.
Radiation therapy to the eyes is given for people with ocular lymphoma.
Surgery
Surgery does not play a role in the treatment of PCNSL because the tumours are often spread throughout the brain and located deep within the brain. A stereotactic biopsy stereotactic biopsy A procedure that uses a 3-dimensional scanning machine ( ultrasound, CT scan or MRI) to find the precise location of a tumour and remove a sample for examination under a microscope. may be done to make a diagnosis.
Recurrent PCNSL
The treatment for recurrent PCNSL depends on the location of the relapse and past treatment. If the person did not receive whole-brain radiation therapy as part of their initial treatment, it may be given for the relapse. Chemotherapy may also be used even if it had been given before, but different drugs may be tried.
Back to top
Treatment in people with immunosuppression
People with AIDS-related PCNSL are treated the same way as people who have a normal immune system, but the treatment is more toxic and can be less effective. AIDS-related PCNSL is often treated with:
anti-HIV drugs, called highly active antiretroviral therapy (HAART) corticosteroids whole-brain radiation therapy (WBRT) Chemotherapy may be given to certain people depending on the status of their immune system.
People who have had organ transplants may need to have their immunosuppressant drug dose decreased or stopped.
Surgical resection is usually ineffective because of the depth of the tumor. Treatment with irradiation and corticosteroids often only produces a partial response, but tumor recurs in more than 90% of patients. Median survival is 10 to 18 months in immunocompetent patients, and less in those with AIDS. The addition of IV methotrexate and citrovorum may extend survival to a median of 3.5 years. If radiation is added to methotrexate, median survival may increase beyond 4 years. However, radiation is not recommended in conjunction with methotrexate because of increased risk of leukoencephalopathy and dementia in patients older than 60 years of age[1].
References
- ↑ Deangelis LM, Hormigo A. Treatment of primary central nervous system lymphoma. Semin Oncol 2004; 31:684-692. In AIDS patients, perhaps the most important factor with respect to treatment is the use of highly active anti-retroviral therapy (HAART), which affects the CD4+ lymphocyte population and the level of immunosuppression