General Information About Primary CNS Lymphoma
Primary central nervous system (CNS) lymphoma is defined as lymphoma limited to the cranial-spinal axis without systemic disease. An increasing incidence of this disease has been seen among patients with AIDS and among other immunocompromised persons. The natural history of this disorder differs between patients with AIDS and those without AIDS. Computed tomographic (CT) scans may show ring enhancement in 50% of AIDS patients while patients without AIDS almost always show only homogeneous enhancement. Median overall survival in published trials generally ranges from 2 to 4 years. Older age (>65 years) and HIV positivity are the most clinically relevant poor prognostic factors.
Poor prognostic factors include the following:[3-5]
- Age older than 50 years.
- Performance status (PS) greater than 1 or Karnofsky PS less than 70.
- Elevated serum level of lactate dehydrogenase.
- Elevated cerebrospinal fluid protein concentration.
- Involvement of nonhemispheric areas of the brain (periventricular, basal ganglia, brainstem, and cerebellum).
- Intraocular disease and concomitant brain involvement.
When tumor progression occurs, it is usually confined to the CNS and/or the eye. Occult systemic disease can be excluded by staging with bone marrow biopsy and CT scans of the chest, abdomen, and pelvis.[7,8]
In one prospective, case series of 282 patients, 17% were found to have meningeal dissemination by cytomorphology, polymerase chain reaction of rearranged, immunoglobulin, heavy-chain genes, or meningeal enhancement on magnetic resonance imaging. The clinical impact of meningeal involvement on prognosis and therapy remains to be evaluated.
Although more than 95% of patients with primary CNS lymphoma have lymphoma of B-cell origin, 45 patients with CNS lymphoma of T-cell origin showed no difference in presentation or outcome in a retrospective series with data collected from 12 cancer centers. Almost all primary CNS lymphomas are aggressive neoplasms of the diffuse large B-cell type. In a retrospective case series derived from 18 cancer centers in five countries of 40 patients with low-grade primary CNS lymphoma, a better long-term outcome was shown (median survival, 7 years) than is associated with the usual aggressive CNS lymphoma.[Level of evidence: 3iiiDiv] Anecdotal cases of primary CNS Hodgkin lymphoma have also been reported.
Other PDQ summaries containing information related to primary CNS lymphoma include:
- Fine HA, Mayer RJ: Primary central nervous system lymphoma. Ann Intern Med 119 (11): 1093-104, 1993. [PUBMED Abstract]
- Grommes C, DeAngelis LM: Primary CNS Lymphoma. J Clin Oncol 35 (21): 2410-2418, 2017. [PUBMED Abstract]
- Ferreri AJ, Blay JY, Reni M, et al.: Prognostic scoring system for primary CNS lymphomas: the International Extranodal Lymphoma Study Group experience. J Clin Oncol 21 (2): 266-72, 2003. [PUBMED Abstract]
- Pollack IF, Lunsford LD, Flickinger JC, et al.: Prognostic factors in the diagnosis and treatment of primary central nervous system lymphoma. Cancer 63 (5): 939-47, 1989. [PUBMED Abstract]
- Abrey LE, Ben-Porat L, Panageas KS, et al.: Primary central nervous system lymphoma: the Memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol 24 (36): 5711-5, 2006. [PUBMED Abstract]
- Kreher S, Strehlow F, Martus P, et al.: Prognostic impact of intraocular involvement in primary CNS lymphoma: experience from the G-PCNSL-SG1 trial. Ann Hematol 94 (3): 409-14, 2015. [PUBMED Abstract]
- O’Neill BP, Dinapoli RP, Kurtin PJ, et al.: Occult systemic non-Hodgkin’s lymphoma (NHL) in patients initially diagnosed as primary central nervous system lymphoma (PCNSL): how much staging is enough? J Neurooncol 25 (1): 67-71, 1995. [PUBMED Abstract]
- Abrey LE, Batchelor TT, Ferreri AJ, et al.: Report of an international workshop to standardize baseline evaluation and response criteria for primary CNS lymphoma. J Clin Oncol 23 (22): 5034-43, 2005. [PUBMED Abstract]
- Fischer L, Martus P, Weller M, et al.: Meningeal dissemination in primary CNS lymphoma: prospective evaluation of 282 patients. Neurology 71 (14): 1102-8, 2008. [PUBMED Abstract]
- Shenkier TN, Blay JY, O’Neill BP, et al.: Primary CNS lymphoma of T-cell origin: a descriptive analysis from the international primary CNS lymphoma collaborative group. J Clin Oncol 23 (10): 2233-9, 2005. [PUBMED Abstract]
- Jahnke K, Korfel A, O’Neill BP, et al.: International study on low-grade primary central nervous system lymphoma. Ann Neurol 59 (5): 755-62, 2006. [PUBMED Abstract]
- Gerstner ER, Abrey LE, Schiff D, et al.: CNS Hodgkin lymphoma. Blood 112 (5): 1658-61, 2008. [PUBMED Abstract]