eMedicine Specialties > Neurology > Neuro-oncology

Primary CNS Lymphoma: Follow-up

Author: Tarakad S Ramachandran, MBBS, FRCP(C), FACP, Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital
Contributor Information and Disclosures

Updated: Jun 22, 2009

Follow-up

Further Inpatient Care

  • After biopsy, some patients may require inpatient care because of neurological deficits related to the primary site of the tumor.
  • The use of corticosteroids to control symptoms may be unavoidable, but administration of dexamethasone or its equivalent, in doses greater than 8 mg/day for more than 2 weeks, raises the risk of steroid myopathy. Patients with muscle weakness due to steroids may require inpatient rehabilitation.
  • High-dose systemic methotrexate must be administered in the hospital.

Further Outpatient Care

  • Recurrence or failure of first-line chemotherapy
    • Patients are monitored with monthly MRI scans after initiation of methotrexate chemotherapy. Those with primary leptomeningeal disease should undergo lumbar puncture with cytologic sampling of CSF at monthly intervals.
    • The management of progressive (ie, methotrexate failure) or recurrent PCNSL is not yet well established. The age and performance status of the patient must be considered. In general, greater than 25% enlargement of previous areas of gadolinium-contrast enhancement, appearance of new contrast-enhancing lesions, or appearance of malignant cells in the CSF, vitreous or, rarely, elsewhere in the body, constitutes treatment failure. Treatment options include the following:
      • Return from monthly (maintenance) to biweekly methotrexate therapy.
      • Consider IV cytarabine.
      • Radiation therapy is probably the best second-line anti-PCNSL treatment.
      • Chemotherapy failure indicates that combined therapy is necessary, accepting that the risk of neurotoxicity from radiation and chemotherapy is considerable. Until the efficacy of repeat intensive methotrexate therapy is established in relapsed disease or until other chemotherapeutic agents are tested more extensively, whole-brain radiation therapy with 4000 cGy in 20-25 daily treatments is recommended.

Inpatient & Outpatient Medications

Although corticosteroids should be avoided during methotrexate therapy, their use may improve quality of life by minimizing symptoms due to vasogenic edema or tumor mass effect; the smallest effective dose should be prescribed.

Deterrence/Prevention

Since the reasons for development of PCNSL in immunocompetent patients are unknown, whether deterrent or preventive maneuvers can be undertaken also is unknown.

  • Patients receiving immunosuppressive therapy after organ transplantation should receive the smallest effective doses compatible with viability of the transplant. Corticosteroid-sparing therapy is advised, and lowering intensity of immunosuppression, if feasible, is advised for transplant recipients who develop PCNSL.
  • Patients with AIDS who have low CD4+ counts are at greatest risk for PCNSL. The extent to which highly effective antiretroviral therapy will affect the incidence and prognosis of PCNSL in AIDS is not yet known.

Complications

  • The major complications of methotrexate therapy are discussed in Medication.
  • Long-term sequelae of radiation therapy and chemotherapy in PCNSL are significant. Although median survival duration has been extended with combined chemotherapy and radiation therapy, the percentage of survivors with late cerebral white-matter toxicity resulting in cognitive dysfunction approaches 50%.
  • Serious leukoencephalopathy also is seen in patients receiving methotrexate chemotherapy alone, but the incidence appears to be lower than that of the cerebral white-matter toxicity seen with combination therapy.

Prognosis

  • Immunocompetent patients
    • The initial response to radiation therapy is excellent, often resulting in complete resolution of radiographic abnormalities. Nevertheless, the duration of response is short, and median survival duration with radiation therapy alone averages only 18 months. Relapse in patients with parenchymal disease is usually within the brain, though leptomeningeal, vitreous and, rarely, systemic recurrences are reported.
    • Methotrexate-based chemotherapy as the sole treatment modality results in median survival duration approaching 48 months.
  • Patients with AIDS
    • Patients with AIDS have only a 4-month median survival duration with radiation therapy. Untreated, such patients often die within 2.5 months, sometimes because of coexisting infections.
    • Extended survival in subgroups of HIV-infected patients with CD4+ counts greater than 200/mm3 and no concurrent opportunistic infections have been reported. With a regimen of intrathecal methotrexate, concurrent systemic procarbazine, cyclohexylchloroethylnitrosurea (CCNU), vincristine, and whole-brain radiotherapy, this small subset of patients with AIDS had median survival durations ranging from 10-18 months.

Patient Education

  • Patients receiving high-dose intravenous methotrexate must be educated carefully about the drugs to be avoided in the week prior to chemotherapy and about the fluid and intensive monitoring requirements of their inpatient stay.
  • Patients who have left the hospital should be educated about the possibility of seizures and should be encouraged to pursue physical therapy to maximize motor function.
  • Patients with AIDS should continue to follow the antiretroviral regimen recommended by their physician.
  • For excellent patient education resources, visit eMedicine's Cancer and Tumors Center. Also, see eMedicine's patient education article Brain Cancer.

Miscellaneous

Medicolegal Pitfalls

  • Since the clinical and neuroimaging presentation of PCNSL can be so varied and the differential diagnostic possibilities are therefore so large, no patient should be treated for PCNSL without definitive cytologic proof of diagnosis, either by vitrectomy, positive CSF cytology, or brain biopsy.
  • Patients with AIDS may have coexisting infections; any change in neurologic examination findings or neuroimaging studies should be accordingly investigated broadly for the possibility of a diagnosis besides PCNSL.
 


More on Primary CNS Lymphoma

Overview: Primary CNS Lymphoma
Differential Diagnoses & Workup: Primary CNS Lymphoma
Treatment & Medication: Primary CNS Lymphoma
Follow-up: Primary CNS Lymphoma
References
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References

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Further Reading

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Keywords

reticulum cell sarcoma, diffuse histiocytic lymphoma, microglioma, high-grade non-Hodgkin B-cell neoplasm, primary CNS lymphoma, primary central nervous system lymphoma, PCNSL, diffuse large B-cell lymphoma, DLBCL, low-grade lymphomas, Burkitt lymphomas, T-cell lymphomas

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Contributor Information and Disclosures

Author

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital
Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine
Disclosure: Abbott Labs  Honoraria Consulting; Teva Marion Honoraria Consulting; Boeringer-Ingelheim Honoraria Speaking and teaching

Medical Editor

Frederick M Vincent Sr, MD, Clinical Professor, Department of Neurology and Ophthalmology, Michigan State University Colleges of Human and Osteopathic Medicine
Frederick M Vincent Sr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Forensic Examiners, American College of Legal Medicine, American College of Physicians, and Michigan State Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Jorge Kattah, MD, Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria
Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences
Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Stephen A Berman, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

 
 
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