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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}}: Mediastinal B-cell lymphoma; Mediastinal large B-cell lymphoma, PMBCL, Primary mediastinal B-cell lymphoma.
| | ==[[Primary mediastinal large B-cell lymphoma overview|Overview]]== |
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| == Overview == | | ==[[Primary mediastinal large B-cell lymphoma historical perspective|Historical Perspective]]== |
| Primary mediastinal large B-cell lymphoma (PMBCL) is a subtype of [[diffuse large B-cell lymphoma]] (DLBCL). It is also considered a distinct type of [[non-Hodgkin lymphoma]] (NHL) in the World Health Organization (WHO) classification system. It occurs in the thymus gland. The small gland in the centre of the chest behind the sternum where lymphocytes mature, multiply and become T cells. or lymph nodes in the center of the chest. On microscopic histopathological analysis, large-sized cells and alveolar fibrosis are characteristic findings of primary mediastinal large B-cell lymphoma. The incidence of primary mediastinal large B-cell lymphoma increases with age; the median age at diagnosis is 35 years. Symptoms of the primary mediastinal large B-cell lymphoma include [[fever]], [[weight loss]], [[night sweats]], skin rash, facial swelling, cough, shortness of breath, and painless swelling in the neck, axilla, groin, thorax, or abdomen. Lymph node or mediastinal mass biopsy is diagnostic of primary mediastinal large B-cell lymphoma. The predominant therapy for primary mediastinal large B-cell lymphoma is [[chemotherapy]]. Adjunctive [[radiotherapy]], [[stem cell transplant]], and [[biological therapy]] may be required. The optimal therapy for primary mediastinal large B-cell lymphoma depends on the clinical presentation.<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><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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| == Pathophysiology== | | ==[[Primary mediastinal large B-cell lymphoma classification|Classification]]== |
| * Primary mediastinal large B-cell lymphoma most likely arises within the thymus.<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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| * Patients present with a localized anterosuperior mediastinal mass.
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| * The mass is often bulky and frequently invades adjacent structures such as lungs, pleura, or pericardium.
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| * Spread to supraclavicular and cervical lymph node can occur.
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| ===Genetics===
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| Genes involved in the pathogenesis of primary mediastinal large B-cell lymphoma include:
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| * Comparative genomic hybridzation demonstrated gains in chromosome 9p24 and 2p15
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| * Genomic hybridization in chromosome X-p11.4-21
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| * Immunoglobulin genes clonally rearranged
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| ===Microscopic Pathology===
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| On microscopic histopathological analysis, large-sized cells and alveolar fibrosis are characteristic findings of primary mediastinal large B-cell lymphoma.
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| <gallery widths=200px>
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| Image:Primary mediastinal large B-cell lymphoma pathology .jpg|Hematoxylin and eosin (50X). Primary mediastinal B cells (PMBC) associated with delicate interstitial fibrosis.<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crihem/2012/197347/. Accessed on March 07, 2016 </ref>
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| Image:Primary medistinal large b-cell lymphoma pathology 1 .jpg| Primary mediastinal large B-cell lymphoma immunoreactivity for B cell antigen on the membrane, CD20<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crihem/2012/197347/. Accessed on March 07, 2016 </ref>
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| Image:Primary mediastinal large B-cell lymphoma pathology 2.jpg|Primary mediastinal large B-cell lymphoma immunoreactivity for CD30<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crihem/2012/197347/. Accessed on March 07, 2016 </ref>
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| </gallery>
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| ==Causes== | | ==[[Primary mediastinal large B-cell lymphoma pathophysiology|Pathophysiology]]== |
| 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.<ref name=uspreventive> Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Primary+mediastinal+large+B-cell+lymphoma+. Accessed on March 7, 2016</ref>
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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 ==
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| ===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 causes|Causes]]== |
| |+ '''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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| | 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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| |-
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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 differential diagnosis|Differentiating Primary mediastinal large B-cell lymphoma from other Diseases]]== |
| 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]]
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| * [[Weight loss]]
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| * [[Night sweats]]
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| * Skin rash
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| * Shortness of breath
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| * Facial swelling
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| * Cough
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| * Painless swelling in the neck, axilla, groin, thorax, and abdomen
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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> === | | ==[[Primary mediastinal large B-cell lymphoma epidemiology and demographics|Epidemiology and Demographics]]== |
| ====Vitals====
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| * [[Fever]] is often present
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| ====Skin====
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| * [[Rash]]
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| ====HEENT====
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| * [[Lymphadenopathy|Cervical lymphadenopathy]]
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| * Facial edema
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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==== | |
| * [[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 === | | ==[[Primary mediastinal large B-cell lymphoma risk factors|Risk Factors]]== |
| 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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| ====Chest X-Ray====
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| Chest X-ray may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma. Finding on chest X-ray suggestive of primary mediastinal large B-cell lymphoma includes large anterior mediastinal mass.<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crionm/2013/814291/. Accessed on March 07, 2016 </ref>
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| ====Biopsy====
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| Lymph node or mediastinal mass biopsy is diagnostic of primary mediastinal large B-cell lymphoma.
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| ====Echocardiography====
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| Echocardiography may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.
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| <gallery widths=200px>
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| Image:Primary mediastinal large B-cell lymphoma echo.jpg|A comprehensive 2 dimensional M-mode color flow and Doppler echocardiography reveals a normal left ventricular systolic function (EF 60–69%). A large right atrial mass measuring cm almost fills the right atrium and extends into the tricuspid valve causing tricuspid regurgitation.<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crionm/2013/814291/. Accessed on March 07, 2016 </ref>
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| </gallery>
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| ====CT ==== | | ==[[Primary mediastinal large B-cell lymphoma screening|Screening]]== |
| [[CT]] scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma. | | |
| <gallery widths=200px>
| | ==[[Primary mediastinal large B-cell lymphoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| Image:Primary mediastinal large B-cell lymphoma CT scan 1 .jpg|CT scan of the chest with contrast reveals a large lobulated anterior mediastinal solid mass (black arrow) with extension into the right hemithorax and the right atrium. There is displacement of the great vessels into the left hemithorax with significant mass effect on the right upper lobe. The tumor causes compression of the right pulmonary artery (red arrow) and right and left mainstem bronchi (white arrows).<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crionm/2013/814291/. Accessed on March 07, 2016 </ref>
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| Image:Primary mediatinsl large B-cell lymphoma coronal CT scan.jpg|Coronal CT scan image elucidates a mediastinal mass with extension into the right atrium (black arrow) with complete encasement and compression of the SVC. The tumor extends to the confluence of the IVC in the right atrium causing dilatation of the intraabdominal IVC and hepatic veins suggesting compromised cardiac return (red arrows). Tumor causes the displacement of great vessels into the left hemithorax.<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crionm/2013/814291/. Accessed on March 07, 2016 </ref>
| | ==Diagnosis== |
| </gallery>
| | [[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]] |
| ==== MRI ====
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| [[MRI]] scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma. | |
| <gallery widths=200px>
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| Image:Primary medistinal large B-cell lymphoma MRI .jpg| Cardiac MRI short axis T1 at the level of mitral valve reveals a large mediastinal mass infiltrating and obliterating the SVC causing SVC obstruction. The tumor extends into the right atrium (red arrow) and invades the tricuspid valve.<ref name=hindawi>Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crionm/2013/814291/. Accessed on March 07, 2016 </ref>
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| </gallery>
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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]] | |
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| * [[Rituximab]] may be added to chemotherapy.
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| | style="padding: 5px 5px; background: #DCDCDC;" | [[Radiation therapy]] | |
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| * [[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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| ==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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