Primary mediastinal large B-cell lymphoma: Difference between revisions
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{{CMG}}; {{AE}} {{AS}} | {{CMG}}; {{AE}} {{AS}} | ||
{{SK}}: Mediastinal B-cell lymphoma; Mediastinal large B-cell lymphoma. | {{SK}}: Mediastinal B-cell lymphoma; Mediastinal large B-cell lymphoma, PMBCL. | ||
== Overview == | == 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 centre of the chest. | |||
== Pathophysiology== | == Pathophysiology== | ||
Revision as of 17:02, 7 March 2016
For patient information, click Insert page name here
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.
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 centre of the chest.
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 node can occur.
Associated Conditions
Microscopic Pathology
On microscopic histopathological analysis, 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.
Natural History, Complications and Prognosis
- Primary mediastinal large B-cell lymphoma is usually a fast-growing (aggressive) lymphoma.
- Often have localized disease in the chest at first.
- Can cause shortness of breath, cough or chest pain as the tumour or mass grows in the chest.
- 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.
- Recurrences or relapses often occur 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:[2]
| 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
- Painless swelling in the neck, axilla, groin, thorax, and abdomen
Physical Examination[1]
Vitals
- Fever is often present
HEENT
Thorax
- Thoracic masses suggestive of central lymphadenopathy
- Localized anterosuperior mediastinal mass
Abdomen
- Abdominal masses suggestive of central lymphadenopathy
Extremities
Laboratory Findings
Laboratory tests for primary mediastinal large B-cell lymphoma include:[1]
- Complete blood count (CBC)
- Blood chemistry studies
- Cytogenetic analysis
- Flow cytometry
- Immunohistochemistry
- Immunophenotyping:
Biopsy
Lymph node or bone marrow biopsy is diagnostic of .
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
| Therapy | Description |
|---|---|
| Chemotherapy |
|
| Biological therapy |
|
| Radiation therapy |
|
| Stem cell transplant |
|
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Primary mediastinal large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5318/. Accessed on March 7, 2016
- ↑ 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. doi:10.1200/JCO.2013.54.8800. ISSN 1527-7755. PMID 25113753.
- ↑ 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