Primary CNS Lymphoma Treatment

Radiographically, the major difference between these two patient populations is that there is approximately twice the incidence of multiple lesions seen at the time of presentation radiographically in AIDS patients and that about half of those lesions were characteristic ring-enhancing lesions in AIDS patients, a radiographic finding that is very uncommon, actually, in immunocompetent patients. Just to give you a typical kind of MRI scan in an AIDS patient with CNS lymphoma; you can see the multiple lesions, you can see the typical ring-enhancing lesion and their distribution in a ventricular manner, with a relatively small amount of associated edema in contrast with what you see in high grade gliomas.
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The diagnosis of primary CNS lymphoma can occasionally be made relatively non-invasively, and that about 10-20% of patients will develop a lymphomatous uveitis that actually can be detected on slit lamp exam, if in fact one is astute enough to suspect primary CNS lymphoma radiographically and have the patient seen by an ophthalmologist. Although lumbar puncture and CNS examination will give you a clear cytosis in approximately 80% of the patients, actual lymphomatous involvement as called by the cytologist is generally found in less than 10% of the cases. And that surgery is still often required to make the diagnosis in this disease. The question often comes up that once one has made the diagnosis of lymphoma in the brain, how much systemic staging should be done. And although this remains an area of somewhat debate and controversy and personal preference, the bottom line is this: that for a patient who presents with neurologic symptoms, has radiographic evidence of a tumor on MRI scan or CAT scan, then goes on to have a biopsy and it is shown to be lymphoma, if you do a good physical exam on those patients, you do a chest x-ray, standard blood work including liver function tests and you do not detect any obvious abnormality, the likelihood of finding an occult systemic lymphoma with the more extensive work-up with tests such as body CT scans, gallium scans, bone marrow biopsies, is essentially zero. Although there may be one in a hundred or more cases that you’ll finally pick up. Because the pick up rate is so small we actually do not recommend doing that type of extensive analysis. So again, our recommendations are: good physical exam, standard blood work, chest x-ray. If there’s no signs of systemic lymphoma then almost by definition they have primary CNS lymphoma and should be treated accordingly.
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What is the treatment for primary CNS lymphoma? Well, opposed to what we talked about for gliomas the role for surgery is much more restricted, mostly for diagnostic purposes. And that extensive resection is really not optimal in this case. One, because of the severely infiltrative nature of this disease, not just locally but throughout the craniospinal axis, but also because the other therapies, radiation therapy and chemotherapy, are so much more effective than they are for astrocytic tumors. The only problem with stereotactic biopsies for this tumor is that sometimes the histologic diagnosis from such a small sample can be difficult, in that the pathologist may say, well we see some lymphocytes but we can’t tell you whether this is an inflammatory lesion or lymphoma. However, increasingly so with molecular diagnostics such as through PCR looking for immunoglobulin gene rearrangements one can often make the diagnosis from very small tissue samples.

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