Primary central nervous system lymphoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Classification
History & Symptoms
Primary CNS lymphoma usually presents with seizures, headache, cranial nerve findings, altered mental status, or other focal neurological deficits typical of a mass effect[1] [2]. Systemic symptoms may include fever, night sweats, or weight loss.
Diagnosis
MRI
CT
Other Diagnostic Studies
MRI or contrast enhanced CT usually shows multiple (1 to 3) 3- to 5-cm ring-enhancing lesions in almost any location, but usually deep in the white matter. The major differential diagnosis is cerebral toxoplasmosis, which is also prevalent in AIDS patients and also presents with a ring-enhanced lesion, although the contrast enhancement is more pronounced in toxoplasmosis and it presents with more lesions.
Because imaging techniques cannot distinguish the two conditions with certainty, patients usually undergo brain biopsy if the lesion is solitary or a trial of toxoplasmosis therapy is non-therapeutic. In the future, it may be possible to use PCR assay of cerebrospinal fluid for EBV DNA.
Treatment
Medical Therapy
Surgical resection is usually ineffective because of the depth of the tumor. Treatment with irradiation and corticosteroids often only produces a partial response, but tumor recurs in more than 90% of patients. Median survival is 10 to 18 months in immunocompetent patients, and less in those with AIDS. The addition of IV methotrexate and citrovorum may extend survival to a median of 3.5 years. If radiation is added to methotrexate, median survival may increase beyond 4 years. However, radiation is not recommended in conjunction with methotrexate because of increased risk of leukoencephalopathy and dementia in patients older than 60 years of age[3].
References
- ↑ Herrlinger U, Schabet M, Bitzer M, Petersen D, Krauseneck P. Primary central nervous system lymphoma: from clinical presentation to diagnosis. J Neurosurg 2000; 92:261-266
- ↑ Herrlinger U, Schabet M, Bitzer M, Petersen D, Krauseneck P. Primary central nervous system lymphoma: from clinical presentation to diagnosis.J.Neurooncol. 1999;43:219-226. (PMID: 10563426).
- ↑ Deangelis LM, Hormigo A. Treatment of primary central nervous system lymphoma. Semin Oncol 2004; 31:684-692. In AIDS patients, perhaps the most important factor with respect to treatment is the use of highly active anti-retroviral therapy (HAART), which affects the CD4+ lymphocyte population and the level of immunosuppression