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::

Pathophysiology

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.

Gender

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

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.

Diagnosis

Staging

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

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:

• Fever
• Weight loss
• Night sweats
• Skin rash
• Painless swelling in the neck, axilla, groin, thorax, and abdomen

Physical Examination

Vitals

• Fever is often present

HEENT

• Cervical lymphadenopathy

Thorax

• Thoracic masses suggestive of central lymphadenopathy
• Localized anterosuperior mediastinal mass

Abdomen

• Abdominal masses suggestive of central lymphadenopathy

Extremities

• Peripheral lymphadenopathy

Laboratory Findings

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

• Complete blood count (CBC)
• Blood chemistry studies
• Cytogenetic analysis
• Flow cytometry
• Immunohistochemistry
• Immunophenotyping:

• Positive: CD19, CD79a, CD20, CD30, and CD22

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

Treatment of primary mediastinal large B-cell lymphoma

Therapy	Description
Chemotherapy
Biological therapy
Radiation therapy
Stem cell transplant