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 acquired immunodeficiency syndrome (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 one half of AIDS patients while patients without AIDS almost always show only homogeneous enhancement.[1] Both groups do equally poorly without therapy (1-3 month mean survival), but the overall survival for treated patients is much better for patients without AIDS (18.9 months) than for those with AIDS (2.6 months).[1,2]

Poor prognostic factors include the following:[3,4]

• Age older than 60 years.
• Performance status over 1.
• Elevated serum level of lactate dehydrogenase.
• Elevated cerebrospinal fluid protein concentration.
• Involvement of nonhemispheric areas of the brain (periventricular, basal ganglia, brainstem, cerebellum).

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.[5]

References

1. Fine HA, Mayer RJ: Primary central nervous system lymphoma. Ann Intern Med 119 (11): 1093-104, 1993.  [PUBMED Abstract]
   
   
2. Nasir S, DeAngelis LM: Update on the management of primary CNS lymphoma. Oncology (Huntingt) 14 (2): 228-34; discussion 237-42, 244, 2000.  [PUBMED Abstract]
   
   
3. 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]
   
   
4. 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]
   
   
5. 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]
   
   

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