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| {{SI}} | | {{Primary mediastinal large B-cell lymphoma}} |
| | '''For patient information, click [Primary mediastinal large B-cell lymphoma(patient information)|here]]''' |
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| '''For patient information, click [[Insert page name here (patient information)|Insert page name here]]'''
| | {{CMG}}; {{AE}} {{Badria}} {{AS}} |
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| {{CMG}}; {{AE}} {{AS}} | | {{SK}} |
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| {{SK}}:
| | ==[[Primary mediastinal large B-cell lymphoma overview|Overview]]== |
| == Overview == | |
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| | ==[[Primary mediastinal large B-cell lymphoma historical perspective|Historical Perspective]]== |
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| == Pathophysiology== | | ==[[Primary mediastinal large B-cell lymphoma classification|Classification]]== |
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| === Associated Conditions=== | | ==[[Primary mediastinal large B-cell lymphoma pathophysiology|Pathophysiology]]== |
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| ===Microscopic Pathology=== | | ==[[Primary mediastinal large B-cell lymphoma causes|Causes]]== |
| On microscopic histopathological analysis, are characteristic findings of primary mediastinal large B-cell lymphoma.
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| ==Causes==
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| There are no established causes for primary mediastinal large B-cell lymphoma.
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| ==Differentiating type page name here from other Diseases==
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| Primary mediastinal large B-cell lymphoma must be differentiated from other diseases such as:
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| * [[Thymoma]]
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| * [[Hodgkin's lymphoma]]
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| * [[Thymic carcinoma]]
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| ==Epidemiology and Demographics==
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| ===Age===
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| The incidence of primary mediastinal large B-cell lymphoma increases with age; the median age at diagnosis is 35 years.<ref name=seer.cancer.gov>Primary mediastinal large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5318/. Accessed on March 7, 2016 </ref>
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| ===Gender===
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| Females are more commonly affected with primary mediastinal large B-cell lymphoma than males.<ref name=seer.cancer.gov>Primary mediastinal large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5318/. Accessed on March 7, 2016 </ref>
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| == Risk Factors ==
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| There are no established risk factors for primary mediastinal large B-cell lymphoma.
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| == Screening ==
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| According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for primary mediastinal large B-cell lymphoma.
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| == Natural History, Complications and Prognosis==
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| * Primary mediastinal large B-cell lymphoma is usually a fast-growing (aggressive) lymphoma.
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| * Often have localized disease in the chest at first.
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| * Can cause shortness of breath, cough or chest pain as the tumour or mass grows in the chest.
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| * Can also partially block the main vein (superior vena cava) that carries blood from the upper body to the heart and cause [[superior vena cava syndrome]].
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| * The bone marrow is rarely affected by this type of lymphoma.
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| * Recurrences or relapses often occur in organs or tissues outside the lymph nodes (extranodal sites), such as the kidneys or central nervous system.
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| == Diagnosis == | | ==[[Primary mediastinal large B-cell lymphoma differential diagnosis|Differentiating Primary mediastinal large B-cell lymphoma from other Diseases]]== |
| ===Staging===
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| Staging for primary mediastinal large B-cell lymphoma is provided in the following table:<ref>{{Cite journal| doi = 10.1200/JCO.2013.54.8800| issn = 1527-7755| volume = 32| issue = 27| pages = 3059–3068| last1 = Cheson| first1 = Bruce D.| last2 = Fisher| first2 = Richard I.| last3 = Barrington| first3 = Sally F.| last4 = Cavalli| first4 = Franco| last5 = Schwartz| first5 = Lawrence H.| last6 = Zucca| first6 = Emanuele| last7 = Lister| first7 = T. Andrew| last8 = Alliance, Australasian Leukaemia and Lymphoma Group| last9 = Eastern Cooperative Oncology Group| last10 = European Mantle Cell Lymphoma Consortium| last11 = Italian Lymphoma Foundation| last12 = European Organisation for Research| last13 = Treatment of Cancer/Dutch Hemato-Oncology Group| last14 = Grupo Español de Médula Ósea| last15 = German High-Grade Lymphoma Study Group| last16 = German Hodgkin's Study Group| last17 = Japanese Lymphorra Study Group| last18 = Lymphoma Study Association| last19 = NCIC Clinical Trials Group| last20 = Nordic Lymphoma Study Group| last21 = Southwest Oncology Group| last22 = United Kingdom National Cancer Research Institute| title = Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification| journal = Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology| date = 2014-09-20| pmid = 25113753}}</ref>
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| {| style="border: 0px; font-size: 90%; margin: 3px;" align=center
| | ==[[Primary mediastinal large B-cell lymphoma epidemiology and demographics|Epidemiology and Demographics]]== |
| |+ '''Revised staging system for primary nodal lymphomas (Lugano classification)'''
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| ! style="background: #4479BA; color:#FFF;" | Stage
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| ! style="background: #4479BA; color:#FFF;" | Involvement
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| ! style="background: #4479BA; color:#FFF;" | Extranodal (E) status
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC;" colspan=3 | '''Limited''' | |
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| | style="padding: 5px 5px; background: #F5F5F5;" | Stage I
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| | style="padding: 5px 5px; background: #F5F5F5;" | One node or a group of adjacent nodes
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| | style="padding: 5px 5px; background: #F5F5F5;" | Single extranodal lesions without nodal involvement
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| |-
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| | style="padding: 5px 5px; background: #F5F5F5;" | Stage II
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| | style="padding: 5px 5px; background: #F5F5F5;" | Two or more nodal groups on the same side of the diaphragm
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| | style="padding: 5px 5px; background: #F5F5F5;" | Stage I or II by nodal extent with limited contiguous extranodal involvement
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| |-
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| | style="padding: 5px 5px; background: #F5F5F5;" | Stage II bulky
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| | style="padding: 5px 5px; background: #F5F5F5;" | II as above with "bulky" disease
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| | style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC;" colspan=3 | '''Advanced'''
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| | style="padding: 5px 5px; background: #F5F5F5;" | Stage III
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| | style="padding: 5px 5px; background: #F5F5F5;" | Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement
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| | style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
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| |-
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| | style="padding: 5px 5px; background: #F5F5F5;" | Stage IV
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| | style="padding: 5px 5px; background: #F5F5F5;" | Additional noncontiguous extralymphatic involvement
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| | style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
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| |}
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| === Symptoms === | | ==[[Primary mediastinal large B-cell lymphoma risk factors|Risk Factors]]== |
| Symptoms of the primary mediastinal large B-cell lymphoma include:<ref name=seer.cancer.gov>Primary mediastinal large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5318/. Accessed on March 7, 2016 </ref>
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| * [[Fever]]
| | ==[[Primary mediastinal large B-cell lymphoma screening|Screening]]== |
| * [[Weight loss]]
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| * [[Night sweats]]
| | ==[[Primary mediastinal large B-cell lymphoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| * Skin rash
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| * Shortness of breath
| | ==Diagnosis== |
| * Painless swelling in the neck, axilla, groin, thorax, and abdomen
| | [[Primary mediastinal large B-cell lymphoma diagnostic study of choice|Diagnostic study of choice]] | [[Primary mediastinal large B-cell lymphoma history and symptoms|History and Symptoms]] | [[Primary mediastinal large B-cell lymphoma physical examination|Physical Examination]] | [[Primary mediastinal large B-cell lymphoma laboratory findings|Laboratory Findings]] | [[Primary mediastinal large B-cell lymphoma electrocardiogram|Electrocardiogram]] | [[Primary mediastinal large B-cell lymphoma x ray|X-Ray Findings]] | [[Primary mediastinal large B-cell lymphoma echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Primary mediastinal large B-cell lymphoma CT scan|CT-Scan Findings]] | [[Primary mediastinal large B-cell lymphoma MRI|MRI Findings]] | [[Primary mediastinal large B-cell lymphoma other imaging findings|Other Imaging Findings]] | [[Primary mediastinal large B-cell lymphoma other diagnostic studies|Other Diagnostic Studies]] |
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| === Physical Examination<ref name=seer.cancer.gov>Primary mediastinal large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5318/. Accessed on March 7, 2016 </ref> ===
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| ====Vitals====
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| * [[Fever]] is often present
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| ====HEENT====
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| * [[Lymphadenopathy|Cervical lymphadenopathy]]
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| ====Thorax====
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| * Thoracic masses suggestive of [[Lymphadenopathy|central lymphadenopathy]]
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| * Localized anterosuperior mediastinal mass
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| ====Abdomen====
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| * [[Abdominal mass]]es suggestive of [[Lymphadenopathy|central lymphadenopathy]]
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| ====Extremities====
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| * [[Lymphadenopathy|Peripheral lymphadenopathy]]
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| ===Laboratory Findings ===
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| Laboratory tests for primary mediastinal large B-cell lymphoma include:<ref name=seer.cancer.gov>Primary mediastinal large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5318/. Accessed on March 7, 2016 </ref>
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| * [[Complete blood count]] (CBC)
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| * Blood chemistry studies
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| * Cytogenetic analysis
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| * [[Flow cytometry]]
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| * [[Immunohistochemistry]]
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| * [[Immunophenotyping]]:
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| :* Positive: [[CD19]], [[CD79a]], [[CD20]], [[CD30]], and [[CD22]]
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| ====Biopsy====
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| Lymph node or bone marrow biopsy is diagnostic of .
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| ====CT ====
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| [[CT]] scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.
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| ==== MRI ====
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| [[MRI]] scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.
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| ==== Other Imaging Findings ====
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| [[PET]] scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy===
| | [[Primary mediastinal large B-cell lymphoma medical therapy|Medical Therapy]] | [[Primary mediastinal large B-cell lymphoma surgery|Surgery]] | [[Primary mediastinal large B-cell lymphoma primary prevention|Primary Prevention]] | [[Primary mediastinal large B-cell lymphoma secondary prevention|Secondary Prevention]] | [[Primary mediastinal large B-cell lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Primary mediastinal large B-cell lymphoma future or investigational therapies|Future or Investigational Therapies]] |
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| {| style="border: 0px; font-size: 90%; margin: 3px;" align=center
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| |+ '''Treatment of primary mediastinal large B-cell lymphoma<ref name= canadiancancer>Primary mediastinal large B-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-mediastinal-large-b-cell-lymphoma/?region=nb. Accessed on March 7, 2016 </ref>
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| ! style="background: #4479BA; color:#FFF;" | Therapy
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| ! style="background: #4479BA; color:#FFF;" | Description
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| | style="padding: 5px 5px; background: #DCDCDC;" | [[Chemotherapy]]
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| * A commonly used combination chemotherapy is CHOP: [[Cyclophosphamide]], [[Doxorubicin]], [[Vincristine]], {{and}} [[Prednisone]]
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| | style="padding: 5px 5px; background: #DCDCDC;" | [[Biological therapy]] | |
| | style="padding: 5px 5px; background: #F5F5F5;" |
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| * [[Rituximab]] may be added to chemotherapy.
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| | style="padding: 5px 5px; background: #DCDCDC;" | [[Radiation therapy]] | |
| | style="padding: 5px 5px; background: #F5F5F5;" | | |
| * [[External beam radiation therapy]] to the mass in the chest may be used after chemotherapy.
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| | style="padding: 5px 5px; background: #DCDCDC;" | [[Stem cell transplant]]
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| * A [[stem cell transplant]] may be offered to some people if their lymphoma returns or relapses after treatment.
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| |} | |
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| ==References== | | ==Case Studies== |
| {{Reflist|2}}
| | [[rimary mediastinal large B-cell lymphoma case study one|Case #1]] |
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| [[Category:Disease]] | | [[Category: (oncology)]] |
| [[Category:Oncology]]
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