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CNS lymphoma may develop at any time in life, from the newborn period to late adulthood. The peak prevalence occurs in the 4th to 5th decades of life, with a separate peak in the 1st decade that reflects the influence of pediatric AIDS. Males tend to be more frequently affected than females by a ratio of almost 2:1.
The clinical signs and symptoms of CNS lymphoma are usually nonspecific. In a series of 352 patients, five clinical forms of presentation were seen: symptoms of a rapidly enlarging intracranial mass (60% of cases), encephalitis (<25%), demyelinating disease (a few cases), stroke (7%), and cranial nerve palsies (a few cases) (47). Very rarely, CNS lymphoma may primarily involve the skull, with extension into the scalp or dura mater (48). Personality changes (especially with thalamic or corpus callosal lesions), cerebellar signs, headaches, seizures, and motor dysfunction were the most common symptoms and signs at presentation in a review of 66 patients with primary CNS lymphoma over a 26-year period at Massachusetts General Hospital (7). In yet another series, patients who had both primary CNS lymphoma and AIDS tended to be younger, had more constitutional symptoms (weight loss, lethargy, night sweats, etc), had worse performance on neurologic tests at the time of clinical presentation, and had a shorter median survival time than patients without AIDS (8).
Analysis of cerebrospinal fluid has yielded variable results in the diagnosis of CNS lymphoma. In the non-AIDS population, cytologic analysis of cerebrospinal fluid is accurate in 4%-43.5% of patients (49). About 25% of AIDS patients have cytologic findings positive for CNS lymphoma (41). Increased amounts of protein and cells (mononuclear, blast, or other lymphoma cells) are commonly noted (49). Elevated opening pressure may also be seen.
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