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, centrocytes, centroblasts, plasma cells, and immunoblasts are characteristic findings of nodal marginal zone 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