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Primary Central Nervous System Lymphoma

Howard A. Fine, MD; and Robert J. Mayer, MD
[+] Article and Author Information

From the Dana-Farber Cancer Institute, The Brain Tumor Center, The Brigham and Women's Hospital, and Harvard Medical School, Boston Massachusetts. Requests for Reprints: Howard A. Fine, MD, Division of Medical Oncology, Dana-Farber Cancer Institute, 44 Binney Street, Boston, MA 02115. Acknowledgments: The authors thank Mary B. Fishman, MD, and George P. Canellos, MD, for review of the manuscript; Alexandra M. Levine, MD, for useful discussions; Rebecca Folkerth, MD, and Jerrold Turner MD, PhD, for contribution of the photomicrographs; and James Suojanen, MD, for the radiographs.

Copyright 2004 by the American College of Physicians

Ann Intern Med. 1993;119(11):1093-1104. doi:10.7326/0003-4819-119-11-199312010-00007
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Objective: To compare the pathogenesis, clinical presentation, therapy, and prognosis of primary central nervous system lymphoma in immunocompetent persons with these characteristics of the disease in patients with AIDS.

Data Sources and Extraction: All English-language papers published between 1980 and 1992 dealing with either lymphoma and the central nervous system or AIDS were reviewed. Patient characteristics, clinical presentation, histologic findings, and treatment and survival data were extracted from each case report and review.

Data Synthesis: Data were available on 792 patients (from 40 reported series) with non-AIDS-associated primary central nervous system lymphoma and 315 patients (from 32 series) with AIDS-associated primary central nervous system lymphoma. Patients with AIDS initially consulted a physician more often when they had global neurologic symptoms compared with patients without AIDS, with more than 50% of the lesions on computed tomographic (CT) scans in patients with AIDS being ring-enhancing and multifocal, a pattern rarely described in immunocompetent patients. The overall survival of the patients without AIDS was 18.9 months compared with 2.6 months for patients with AIDS, with substantial differences remaining even for subgroups of patients similarly treated with radiation and chemotherapy.

Conclusion: Primary central nervous system lymphoma is probably a substantially different disease in persons with and without AIDS with regard to patient characteristics, clinical and radiographic presentation, and prognosis. Recent advances in the treatment of this disease in patients without AIDS have not largely affected patients with AIDS. Substantial improvements in survival in these patients await advances in controlling their human immunodeficiency virus-associated disease.


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Figure 1.
Contrast-enhanced computed tomographic scan of primary central nervous system lymphoma in an immunocompetent host. Top.Bottom.

Before therapy, this scan suggested primary central nervous system lymphoma because of the brightly enhancing, multifocal, periventricular appearance of these lesions, with less mass effect than might be expected from other primary brain tumors or metastases. A scan of the same patient, obtained after just three cycles of systemic chemotherapy, shows a complete radiographic response. At the time of this scan, the patient no longer required glucocorticoids to control cerebral edema and was neurologically asymptomatic.

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Figure 2.
Histologic sections of a large cell primary central nervous system lymphoma. Top.Bottom left.Bottom right.

The tumor is characterized by angiocentricity (tendency to aggregate around small blood vessels), as well as clumped, peripherally located nuclear chromatin, and prominent nucleoli. (Hematoxylin and eosin; original magnification, 400.) This section is stained for glial fibrillary acidic protein, a known intermediate filament found exclusively in cells of astrocytic origin. The darkly staining cells with the elongated processes are reactive astrocytes, often found in pathologic areas (tumor, infection, ischemia) within the cerebrum. (Original magnification, 400.) This tumor section is stained for leukocyte common antigen, a protein found on the surface of almost all cells of lymphocytic origin. The darkly staining cells are the tumor cells, proving the lymphocytic nature of this neoplasms. (Original magnification, 400.).

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Figure 3.
Diagnostic algorithms for patients found to have head computed tomographic scans or magnetic resonance imaging showing lesions consistent with primary central nervous system lymphoma.Top.Bottom.

Diagnostic algorithm for immunocompetent patients seronegative for the human immunodeficiency virus. Diagnostic algorithm for patients seropositive for the human immunodeficiency virus. CBC = complete blood count; CT = computed tomography; HIV = lumbar immunodeficiency virus; LFTs = liver function tests; LP = lumbar puncture; MRI = magnetic resonance imaging; R/O = rule out; PCNSL = primary central nervous system lymphoma.

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