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Three percent of AIDS patients will develop this tumor either prior to AIDS diagnosis or during their subsequent course. In addition to acquired immunosuppression, patients with inherited disorders (such as Wiskott-Aldrich syndrome, severe combined immunodeficiency, and X-linked immunodeficiency) and other acquired disorders of the immune system are predisposed to the development of CNS lymphoma. Immunological studies have suggested a role for Epstein-Barr virus in the production of this tumor. Although subtypes exist, non-Hodgkin's lymphoma of the CNS most commonly consists of histiocytic cells or large immunoblastic cells bearing B cell surface markers in close proximity to the lateral and third ventricles. Sixty percent of these deposits are multiple, and subarachnoid invasion is seen in one-quarter of patients. Vitreous involvement of the eye occurring prior to and during the course of CNS lymphoma has been noted in up to 25% of patients. The involvement of multiple areas of the neuraxis, the eye, and multiple intracranial sites often occurs in the absence of obvious systemic lymphoma. Therapeutic trials of brain radiation therapy are associated with median survivals of less than 1 year. Uniform complete responses of intracranial deposits are recorded following chemotherapy with high-dose intravenous methotrexate, CHOP (cyclophosphamide, hydroxydaunomycin\/doxorubicin, Oncovin (vincristine), and prednisone), high-dose cytosine arabinoside, and intra-arterial methotrexate with barrier modification.<\/jats:p>","DOI":"10.3171\/jns.1988.68.6.0835","type":"journal-article","created":{"date-parts":[[2009,5,8]],"date-time":"2009-05-08T16:14:12Z","timestamp":1241799252000},"page":"835-853","source":"Crossref","is-referenced-by-count":574,"title":["Primary central nervous system lymphoma"],"prefix":"10.3171","volume":"68","author":[{"given":"Fred H.","family":"Hochberg","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas C.","family":"Miller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1002\/path.1700910210","volume":"91","author":"Adams JH","year":"1966","journal-title":"J Pathol"},{"key":"b2","first-page":"130","volume":"26","author":"Agbi CB","year":"1983","journal-title":"Neurochirurgia (Stuttg)"},{"key":"b3","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1182\/blood.V61.3.469.469","volume":"61","author":"Aisenberg AC","year":"1983","journal-title":"Blood"},{"key":"b4","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1007\/BF01406353","volume":"59","author":"Algers G","year":"1981","journal-title":"Acta Neurochir"},{"key":"b5","doi-asserted-by":"publisher","DOI":"10.1002\/jso.2930260209"},{"key":"b6","first-page":"492","volume":"9","author":"Allan PM","year":"1983","journal-title":"Neuropathol Appl Neurobiol"},{"key":"b7","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1084\/jem.159.1.208","volume":"159","author":"\u00c5man P","year":"1984","journal-title":"J Exp Med"},{"key":"b8","first-page":"345","volume":"109","author":"Amberson JB","year":"1985","journal-title":"Arch Pathol Lab Med"},{"key":"b9","first-page":"1","volume":"5","author":"Anders K","year":"1986","journal-title":"Clin Neuropathol"},{"key":"b10","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1093\/infdis\/148.6.967","volume":"148","author":"Andiman W","year":"1983","journal-title":"J Infect Dis"},{"key":"b11","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1126\/science.3898368","volume":"230","author":"Angeletti RH","year":"1985","journal-title":"Science"},{"key":"b12","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1097\/00005072-198305000-00155","volume":"42","author":"Aschenbrener CA","year":"1983","journal-title":"J Neuropathol Exp Neurol"},{"key":"b13","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1001\/archsurg.1929.01140130449031","volume":"18","author":"Bailey P","year":"1929","journal-title":"Arch Surg"},{"key":"b14","doi-asserted-by":"crossref","first-page":"2386","DOI":"10.1002\/1097-0142(197706)39:6<2386::AID-CNCR2820390612>3.0.CO;2-X","volume":"39","author":"Bale JF","year":"1977","journal-title":"Cancer"},{"key":"b15","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1136\/jnnp.37.8.966","volume":"37","author":"Barnett LB","year":"1974","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"b16","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1007\/BF01760633","volume":"152","author":"Benedek L","year":"1941","journal-title":"Dtsch Z Nervehnheilk"},{"key":"b17","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1980.53.5.0714"},{"key":"b18","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1093\/milmed\/99.5.594","volume":"99","author":"Bennett WA","year":"1946","journal-title":"Milit Surg"},{"key":"b19","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/0360-3016(81)90060-2","volume":"7","author":"Berry MP","year":"1981","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"b20","doi-asserted-by":"crossref","first-page":"3348","DOI":"10.1523\/JNEUROSCI.06-11-03348.1986","volume":"6","author":"Bhat S","year":"1986","journal-title":"J Neurosci"},{"key":"b21","first-page":"1","volume-title":"Ataxia-Telangiectasia. 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