Primary mediastinal large B-cell lymphoma

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

Synonyms and Keywords: : Mediastinal B-cell lymphoma; Mediastinal large B-cell lymphoma, PMBCL, Primary mediastinal B-cell lymphoma.

Overview

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.[1][2]

Pathophysiology

  • Primary mediastinal large B-cell lymphoma most likely arises within the thymus.[1]
  • Patients present with a localized anterosuperior mediastinal mass.
  • The mass is often bulky and frequently invades adjacent structures such as lungs, pleura, or pericardium.
  • Spread to supraclavicular and cervical lymph nodes can occur.

Genetics

Genes involved in the pathogenesis of primary mediastinal large B-cell lymphoma include:

  • Comparative genomic hybridzation demonstrated gains in chromosome 9p24 and 2p15
  • Genomic hybridization in chromosome X-p11.4-21
  • Immunoglobulin genes clonally rearranged

Microscopic Pathology

On microscopic histopathological analysis, large-sized cells and alveolar fibrosis are characteristic findings of primary mediastinal large B-cell lymphoma.

Causes

There are no established causes for primary mediastinal large B-cell lymphoma.

Differentiating type page name here from other Diseases

Primary mediastinal large B-cell lymphoma must be differentiated from other diseases such as:

Epidemiology and Demographics

Age

The incidence of primary mediastinal large B-cell lymphoma increases with age; the median age at diagnosis is 35 years.[1]

Gender

Females are more commonly affected with primary mediastinal large B-cell lymphoma than males.[1]

Risk Factors

There are no established risk factors for primary mediastinal large B-cell lymphoma.

Screening

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.[4]

Natural History, Complications and Prognosis

  • Primary mediastinal large B-cell lymphoma is usually a fast-growing (aggressive) lymphoma.
  • Patients often have localized disease in the chest at first.
  • If left untreated, primary mediastinal large B-cell lymphoma can cause shortness of breath, cough, or chest pain as the mass grows in the chest.
  • Primary mediastinal large B-cell lymphoma 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.
  • The bone marrow is rarely affected by this type of lymphoma.
  • Recurrence or relapse often occurs in organs or tissues outside the lymph nodes (extranodal sites), such as the kidneys or central nervous system.

Diagnosis

Staging

Staging for primary mediastinal large B-cell lymphoma is provided in the following table:[5]

Revised staging system for primary nodal lymphomas (Lugano classification)
Stage Involvement Extranodal (E) status
Limited
Stage I One node or a group of adjacent nodes Single extranodal lesions without nodal involvement
Stage II Two or more nodal groups on the same side of the diaphragm Stage I or II by nodal extent with limited contiguous extranodal involvement
Stage II bulky II as above with "bulky" disease Not applicable
Advanced
Stage III Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement Not applicable
Stage IV Additional noncontiguous extralymphatic involvement Not applicable

Symptoms

Symptoms of the primary mediastinal large B-cell lymphoma include:[1]

  • Fever
  • Weight loss
  • Night sweats
  • Skin rash
  • Shortness of breath
  • Facial swelling
  • Cough
  • Painless swelling in the neck, axilla, groin, thorax, and abdomen

Physical Examination[1]

Vitals

Skin

HEENT

Thorax

Abdomen

Extremities

Laboratory Findings

Laboratory tests for primary mediastinal large B-cell lymphoma include:[1]

Chest X-Ray

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.[3]

Biopsy

Lymph node or mediastinal mass biopsy is diagnostic of primary mediastinal large B-cell lymphoma.

Echocardiography

Echocardiography may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.

CT

CT scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.

MRI

MRI scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.

Other Imaging Findings

PET scan may be helpful in the diagnosis of primary mediastinal large B-cell lymphoma.

Treatment

Medical Therapy

Treatment of primary mediastinal large B-cell lymphoma[2]
Therapy Description
Chemotherapy
Biological therapy
Radiation therapy
Stem cell transplant
  • A stem cell transplant may be offered to some people if their lymphoma returns or relapses after treatment.

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Primary mediastinal large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5318/. Accessed on March 7, 2016
  2. 2.0 2.1 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
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Primary mediastinal large B-cell lymphoma.Hindawi Publishing Corporation. http://www.hindawi.com/journals/crihem/2012/197347/. Accessed on March 07, 2016
  4. 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
  5. Cheson, Bruce D.; Fisher, Richard I.; Barrington, Sally F.; Cavalli, Franco; Schwartz, Lawrence H.; Zucca, Emanuele; Lister, T. Andrew; Alliance, Australasian Leukaemia and Lymphoma Group; Eastern Cooperative Oncology Group; European Mantle Cell Lymphoma Consortium; Italian Lymphoma Foundation; European Organisation for Research; Treatment of Cancer/Dutch Hemato-Oncology Group; Grupo Español de Médula Ósea; German High-Grade Lymphoma Study Group; German Hodgkin's Study Group; Japanese Lymphorra Study Group; Lymphoma Study Association; NCIC Clinical Trials Group; Nordic Lymphoma Study Group; Southwest Oncology Group; United Kingdom National Cancer Research Institute (2014-09-20). "Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification". Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology. 32 (27): 3059–3068. ISSN 1527-7755. PMID 25113753. doi:10.1200/JCO.2013.54.8800. 

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