Primary central nervous system lymphoma: Difference between revisions

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Latest revision as of 21:59, 4 April 2019

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Synonyms and keywords: Primary CNS lymphoma; Primary central nervous system lymphomas; Primary CNS lymphomas; Primary lymphoma of the brain; Primary lymphoma of brain; PCNSL; PCNSLs; Microglioma; Diffuse histiocytic lymphoma; Lymphomatosis cerebri; Primary brain lymphoma; Primary diffuse large B-cell lymphoma of the central nervous system; Primary DLBCL of the central nervous system; Primary DLBCL of the CNS; DLBCL-CNS; Primary intraocular lymphoma; Lymphoid malignancy; Extranodal lymphoma

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Primary Central Nervous System Lymphoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Treatment

Case Studies

Case #1

Template:WikiDoc Sources

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-{{DiseaseDisorder infobox |
+__NOTOC__
- Name = Primary central nervous system lymphoma|
+'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
-ICD10 = 85.1 |
+{{Primary central nervous system lymphoma}}
-ICD9 = {{ICD9|200.0}} {{ICD9|202.8}}|
+{{CMG}}{{AE}}{{Marjan}}
-}}
-{{SI}}
+{{SK}} Primary CNS lymphoma; Primary central nervous system lymphomas; Primary CNS lymphomas; Primary lymphoma of the brain; Primary lymphoma of brain; PCNSL; PCNSLs; Microglioma; Diffuse
-{{CMG}}
+histiocytic lymphoma; Lymphomatosis cerebri; Primary brain lymphoma; Primary diffuse large B-cell lymphoma of the central nervous system; Primary DLBCL of the central nervous system; Primary DLBCL of the CNS; DLBCL-CNS; Primary intraocular lymphoma; Lymphoid malignancy; Extranodal lymphoma
 ==[[Primary central nervous system lymphoma overview|Overview]]==
-'''Primary CNS lymphoma''' is a [[brain tumor|primary intracranial tumor]] usually present in those with severe immunosuppression --- commonly in those with [[AIDS]] --- and represents around 20% of all cases of lymphomas in HIV infection (other types being [[Burkitt's lymphoma]] and immunoblastic lymphoma). Primary CNS lymphoma (PCNSL) is highly associated with [[Epstein-Barr virus]] infection (> 90%) in immunodeficient patients<ref>Fine HA, Mayer RJ. Primary central nervous system lymphoma. ''Ann Intern Med'' 1993; 119(11):1093-1104</ref> (such as those with AIDS and those iatrogenically immunosupressed) and does not have predilections for any age group. Mean [[CD4]]+ count at time of diagnosis is ~50/uL. Because of the severity of immunosuppression at the time of diagnosis, it is to no surprise that prognosis is usually poor.  In immunocompetent patients (that is, patients that do not have AIDS or some other immunodeficiency) there is rarely an association with EBV infection or other infectious DNAs.  In the immunocompetent population, PCNSL typically affects older patients in their 50's and 60's.  Importantly, the incidence of PCNSL in the immunocompetent population has been reported to have increased more than 10-fold from 2.5 cases to 30 cases per 10 million population<ref>Eby NL, Grufferman S, Flannelly CM, Schold SC, Jr., Vogel FS, Burger PC. Increasing incidence of primary brain lymphoma in the US. ''Cancer'' 1988;62(11):2461-2465</ref><ref>Corn BW, Marcus SM, Topham A, Hauck W, Curran WJ, Jr. Will primary central nervous system lymphoma be the most frequent brain tumor diagnosed in the year 2000? ''Cancer'' 1997;79(12):2409-2413</ref>.  The cause for the increase in incidence of this disease in the immunocompetent population is unknown.
+==[[Primary central nervous system lymphoma historical perspective|Historical Perspective]]==
 ==[[Primary central nervous system lymphoma classification|Classification]]==
-Most PCNSLs are diffuse large B-cell non-Hodgkin's lymphoma<ref>Lukes RJ, Collins RD. Immunologic characterization of human malignant lymphomas. ''Cancer'' 1974;34:1488-1503</ref><ref>Jellinger K, Radaskiewictz T, Slowik F. Primary malignant lymphomas of the central nervous system in man. ''Acta Neuropathol'' 1975;95-102 (suppl 6)</ref>.
-==[[Primary central nervous system lymphoma history and symptoms|History & Symptoms]]==
+==[[Primary central nervous system lymphoma pathophysiology|Pathophysiology]]==
-Primary CNS lymphoma usually presents with [[seizures]], [[headache]], cranial nerve findings, altered mental status, or other focal neurological deficits typical of a mass effect<ref>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</ref> <ref> 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).</ref>.  Systemic symptoms may include fever, night sweats, or weight loss.
-==Diagnosis==
+==[[Primary central nervous system lymphoma causes|Causes]]==
+==[[Primary central nervous system lymphoma differential diagnosis|Differentiating Primary Central Nervous System Lymphoma from other Diseases]]==
+==[[Primary central nervous system lymphoma epidemiology and demographics|Epidemiology and Demographics]]==
+==[[Primary central nervous system lymphoma risk factors|Risk Factors]]==
-==[[Primary central nervous system lymphoma MRI|MRI]]==
+==[[Primary central nervous system lymphoma screening|Screening]]==
-==[[Primary central nervous system lymphoma CT|CT]]==
+==[[Primary central nervous system lymphoma natural history, complications, and prognosis|Natural History, Complications and Prognosis]]==
-==[[Primary central nervous system lymphoma other diagnostic studies|Other Diagnostic Studies]]==
+==Diagnosis==
-[[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 [[polymerase chain reaction|PCR]] assay of cerebrospinal fluid for EBV DNA.
+[[Primary central nervous system lymphoma staging|Staging]] | [[Primary central nervous system lymphoma history and symptoms|History and Symptoms]] | [[ Primary central nervous system lymphoma physical examination|Physical Examination]] | [[Primary central nervous system lymphoma laboratory tests|Laboratory Findings]] | [[Primary central nervous system lymphoma CT|CT]] | [[Primary central nervous system lymphoma MRI|MRI]] | [[Primary central nervous system lymphoma other imaging findings|Other Imaging Findings]] | [[Primary central nervous system lymphoma other diagnostic studies|Other Diagnostic Studies]]
 ==Treatment==
-==[[Primary central nervous system lymphoma medical therapy|Medical Therapy]]==
+[[Primary central nervous system lymphoma medical therapy|Medical Therapy]] | [[Primary central nervous system lymphoma surgery |Surgery]] | [[Primary central nervous system lymphoma primary prevention|Primary Prevention]] | [[Primary central nervous system lymphoma secondary prevention|Secondary Prevention]] | [[Primary central nervous system lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |  [[Primary central nervous system lymphoma future or investigational therapies|Future or Investigational Therapies]]
-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<ref> 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</ref>.
-==References==
+==Case Studies==
-<references/>
+[[Primary central nervous system lymphoma case study one|Case #1]]
+[[Category:Disease]]
 [[Category:Neurology]]
 [[Category:Neurosurgery]]
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+[[Category:Oncology]]
+[[Category:Medicine]]
+[[Category:Neurology]]
+[[Category:Neurosurgery]]
+[[Category:Hematology]]

Primary central nervous system lymphoma: Difference between revisions

Latest revision as of 21:59, 4 April 2019

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