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{{Primary central nervous system lymphoma}}
{{Primary central nervous system lymphoma}}
{{CMG}}{{AE}}{{SR}}
{{CMG}}{{AE}}{{Marjan}}


==Overview==
==Overview==
'''Primary CNS lymphoma''' is a [[brain tumor|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).
The treatment of primary central nervous system lymphoma depends on the patient’s age, [[performance status]], stage and location of the lymphoma, and whether or not the individual is immunosuppressed. [[Chemotherapy]] is the mainstay of treatment for primary central nervous system lymphoma. [[Corticosteroid|Corticosteroids]] are often used to destroy [[lymphoma]] [[Cells (biology)|cells]] and relieve [[cerebral]] [[edema]] [[radiotherapy|Radiation therapy]] may be given on its own or along with [[chemotherapy]].


==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).
*The treatment of primary central nervous system lymphoma depends on the patient’s age, performance status, stage and location of the lymphoma, and whether or not the individual is immunosuppressed.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref><ref name="Certainfactorsaffectprognosischancofrecoveryandtreatmentoptions1">Certain factors affect prognosis (chance of recovery) and treatment options. National cancer institute 2016. http://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq. Accessed on February 19, 2016</ref>
*Treatment of primary central nervous system lymphoma works best when the tumor has not spread outside the cerebrum and the patient is younger than 60 years, able to carry out most daily activities, and does not have AIDS or immunosuppressive disorders.<ref name="Certainfactorsaffectprognosischancofrecoveryandtreatmentoptions1">Certain factors affect prognosis (chance of recovery) and treatment options. National cancer institute 2016. http://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq. Accessed on February 19, 2016</ref>


Corticosteroids
===Corticosteroids===
*Corticosteroids are often used to treat primary central nervous system lymphoma.
*When primary CNS lymphoma is suspected, corticosteroids are usually not given until after diagnostic tests are done because these drugs can significantly decrease the size of tumors and affect test results.
*Drugs like [[prednisone]] are used to:<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
:*destroy lymphoma cells
:*relieve edema of the brain tissue


Corticosteroids
===Chemotherapy===
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
*[[Chemotherapy]] is the mainstay of treatment for primary central nervous system lymphoma. The chemotherapy drugs commonly used for [[non-Hodgkin lymphoma]] are not effective against primary central nervous system lymphoma because these drugs do not reach the brain or cross the blood-brain barrier. Chemotherapy drugs used to treat brain tumors because they can cross the blood-brain barrier include:<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
relieve swelling (edema) of the brain tissue
:*[[methotrexate|High-dose methotrexate]] with [[leucovorin]] (folinic acid) rescue
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.
::*Methotrexate is an important chemotherapy drug for treating primary central nervous system lymphoma.
::*It may be used alone or in combination with other chemotherapy drugs.
::*It is given intravenously.
:*[[cytarabine|High dose cytarabine]]
*Primary central nervous system lymphoma may also be treated with [[intrathecal chemotherapy]] or with intraventricular chemotherapy via an Ommaya reservoir, 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.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
*[[Chemotherapy]] by itself may be used for elderly people with primary central nervous system lymphoma.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>


Chemotherapy
===Radiation Therapy===
*[[External beam radiation therapy]] may also be offered for primary central nervous system lymphoma.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
*[[Radiation therapy]] is given to the entire brain ('''whole-brain radiation therapy or WBRT''').
*Primary central nervous system lymphoma is sensitive to [[radiation therapy]], however patients treated with radiotherapy alone have 5-year overall survivals of 3-4% and more than 80% relapsed within 10-14 months.<ref name="Yamanaka2013">{{cite journal|last1=Yamanaka|first1=Ryuya|title=Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research|year=2013|doi=10.5772/52757}}</ref>
*Standard radiotherapy for patients consists of 40 Gy to whole brain with an additional boost of 10-20 Gy on the tumor bed.<ref name="Yamanaka2013">{{cite journal|last1=Yamanaka|first1=Ryuya|title=Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research|year=2013|doi=10.5772/52757}}</ref>
*Shibamoto et al. reported the recent improved results of radiation monotherapy, 5-year survival was 25% for patients 63 years old or younger, and 9.8% for those older than 63 years.
*Since total irradiation dose is an important predictor of delayed neurotoxicity, a decrease in the incidence of this complication should be expected if the total irradiation dose is reduced.<ref name="Yamanaka2013">{{cite journal|last1=Yamanaka|first1=Ryuya|title=Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research|year=2013|doi=10.5772/52757}}</ref>
*The combination of radiation therapy and chemotherapy can cause severe damage to the nervous system (neurotoxicity), especially in elderly individuals with primary CNS lymphoma. This can result in changes in [[cognition|cognitive functioning]], [[dementia]], behaviour changes, [[ataxia|balance and coordination problems]], and other neurologic problems.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>


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:
==Treatment of Recurrent Primary Central Nervous System Lymphoma==
*The treatment for recurrent primary central nervous system lymphoma depends on the location of the relapse and past treatment.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
*If the individual 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.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>


high-dose methotrexate with leucovorin (folinic acid) rescue
==Treatment in individuals with Immunosuppression==
Methotrexate is an important chemotherapy drug for treating PCNSL.
*Individuals with AIDS-related primary central nervous system lymphoma are treated identically to the individuals having a normal immune system, but the treatment is more toxic and can be less effective.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
It may be used alone or in combination with other chemotherapy drugs.
*AIDS-related primary central nervous system lymphoma is often treated with:<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
It is given into a vein (intravenously).
:*[[Highly active antiretroviral therapy]] (HAART)
high dose cytarabine (Cytosar, Ara-C)
:*[[Corticosteroids]]
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
:*[[Whole-brain radiation therapy]] (WBRT)
Ommaya reservoir
*[[Chemotherapy]] may be given to certain individuals depending on the status of their immune system.
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.
*Individuals who have had organ transplants may need to have their immunosuppressant drug dose decreased or stopped.<ref name="treatmentofprimarycentralnervoussystemlymphoma1">Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016</ref>
 
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<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>.


==References==
==References==
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Latest revision as of 15:00, 21 May 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

The treatment of primary central nervous system lymphoma depends on the patient’s age, performance status, stage and location of the lymphoma, and whether or not the individual is immunosuppressed. Chemotherapy is the mainstay of treatment for primary central nervous system lymphoma. Corticosteroids are often used to destroy lymphoma cells and relieve cerebral edema Radiation therapy may be given on its own or along with chemotherapy.

Medical Therapy

  • The treatment of primary central nervous system lymphoma depends on the patient’s age, performance status, stage and location of the lymphoma, and whether or not the individual is immunosuppressed.[1][2]
  • Treatment of primary central nervous system lymphoma works best when the tumor has not spread outside the cerebrum and the patient is younger than 60 years, able to carry out most daily activities, and does not have AIDS or immunosuppressive disorders.[2]

Corticosteroids

  • Corticosteroids are often used to treat primary central nervous system lymphoma.
  • When primary CNS lymphoma is suspected, corticosteroids are usually not given until after diagnostic tests are done because these drugs can significantly decrease the size of tumors and affect test results.
  • Drugs like prednisone are used to:[1]
  • destroy lymphoma cells
  • relieve edema of the brain tissue

Chemotherapy

  • Chemotherapy is the mainstay of treatment for primary central nervous system lymphoma. The chemotherapy drugs commonly used for non-Hodgkin lymphoma are not effective against primary central nervous system lymphoma because these drugs do not reach the brain or cross the blood-brain barrier. Chemotherapy drugs used to treat brain tumors because they can cross the blood-brain barrier include:[1]
  • Methotrexate is an important chemotherapy drug for treating primary central nervous system lymphoma.
  • It may be used alone or in combination with other chemotherapy drugs.
  • It is given intravenously.
  • Primary central nervous system lymphoma may also be treated with intrathecal chemotherapy or with intraventricular chemotherapy via an Ommaya reservoir, 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.[1]
  • Chemotherapy by itself may be used for elderly people with primary central nervous system lymphoma.[1]

Radiation Therapy

  • External beam radiation therapy may also be offered for primary central nervous system lymphoma.[1]
  • Radiation therapy is given to the entire brain (whole-brain radiation therapy or WBRT).
  • Primary central nervous system lymphoma is sensitive to radiation therapy, however patients treated with radiotherapy alone have 5-year overall survivals of 3-4% and more than 80% relapsed within 10-14 months.[3]
  • Standard radiotherapy for patients consists of 40 Gy to whole brain with an additional boost of 10-20 Gy on the tumor bed.[3]
  • Shibamoto et al. reported the recent improved results of radiation monotherapy, 5-year survival was 25% for patients 63 years old or younger, and 9.8% for those older than 63 years.
  • Since total irradiation dose is an important predictor of delayed neurotoxicity, a decrease in the incidence of this complication should be expected if the total irradiation dose is reduced.[3]
  • The combination of radiation therapy and chemotherapy can cause severe damage to the nervous system (neurotoxicity), especially in elderly individuals with primary CNS lymphoma. This can result in changes in cognitive functioning, dementia, behaviour changes, balance and coordination problems, and other neurologic problems.[1]

Treatment of Recurrent Primary Central Nervous System Lymphoma

  • The treatment for recurrent primary central nervous system lymphoma depends on the location of the relapse and past treatment.[1]
  • If the individual 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.[1]

Treatment in individuals with Immunosuppression

  • Individuals with AIDS-related primary central nervous system lymphoma are treated identically to the individuals having a normal immune system, but the treatment is more toxic and can be less effective.[1]
  • AIDS-related primary central nervous system lymphoma is often treated with:[1]
  • Chemotherapy may be given to certain individuals depending on the status of their immune system.
  • Individuals who have had organ transplants may need to have their immunosuppressant drug dose decreased or stopped.[1]

References

  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 Treatment of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016
  2. ↑ 2.0 2.1 Certain factors affect prognosis (chance of recovery) and treatment options. National cancer institute 2016. http://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq. Accessed on February 19, 2016
  3. ↑ 3.0 3.1 3.2 Yamanaka, Ryuya (2013). "Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research". doi:10.5772/52757.


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