Primary central nervous system lymphoma natural history, complications, and prognosis
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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
Natural History
- Primary central nervous system lymphoma is usually a fast-growing (aggressive) lymphoma.[1]
- Primary central nervous system lymphoma often recurs after treatment.
- The clinical course is rapidly fatal; these patients usually present signs of elevated intracranial pressure, nausea, papilledema, vomiting, and neuropsychiatric symptoms.[2]
Complications
Prognosis
- The prognosis of primary central nervous system lymphoma is generally poor.[1]
- Poor prognostic factors for primary central nervous system lymphoma include:[1]
- Elderly patient (>50 years)
- Poor performance status of the individual (PS > 1 or Karnofsky PS < 70)
- AIDS-related primary central nervous system lymphoma
- Elevated serum level of lactate dehydrogenase
- Elevated cerebrospinal fluid protein concentration
- Involvement of nonhemispheric areas of the brain (periventricular, basal ganglia, brainstem, and cerebellum)
In Immunocompetent Patients
- The initial response to radiotherapy is often excellent, and may result in a complete remission. However, the duration of response with radiotherapy alone remains short, with median survival after treatment with radiotherapy is 18 months.
- Methotrexate-based chemotherapy markedly improves survival, with some studies showing median survival after methotrexate chemotherapy reaching 48 months.[3]
In AIDS patients
- Patients with AIDS and primary central nervous system lymphoma have a median survival of only 4 months with radiotherapy alone. Untreated, median survival is only 2.5 months, sometimes due to concurrent opportunistic infections rather than the lymphoma itself.[3]
- Extended survival has been seen, however, in a subgroup of AIDS patients with CD4 counts of more than 200 and no concurrent opportunistic infections, who can tolerate aggressive therapy consisting of either methotrexate monotherapy or vincristine, procarbazine, or whole brain radiotherapy.
- These patients have a median survival of 10–18 months. Of course, highly active antiretroviral therapy (HAART) is critical for prolonged survival in any AIDS patient, so compliance with HAART may play a role in survival in patients with concurrent AIDS and primary CNS lymphoma.[3]
References
- ↑ 1.0 1.1 1.2 Prognosis 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
- ↑ Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). "A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment". Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
- ↑ 3.0 3.1 3.2 Prognosis of primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016