Primary mediastinal B-cell lymphoma

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Primary mediastinal B-cell lymphoma
Micrograph of primary mediastinal large B-cell lymphoma
Synonyms N/A
Pronounce N/A
Specialty N/A
Symptoms Cough, chest pain, dyspnea
Complications Superior vena cava syndrome, pleural effusion
Onset Typically in young adults
Duration Variable
Types N/A
Causes Unknown
Risks Female gender, young adulthood
Diagnosis Biopsy, imaging studies
Differential diagnosis Hodgkin lymphoma, thymoma, germ cell tumor
Prevention N/A
Treatment Chemotherapy, radiation therapy
Medication N/A
Prognosis Generally favorable with treatment
Frequency Rare
Deaths N/A


A type of non-Hodgkin lymphoma


Primary mediastinal B-cell lymphoma (PMBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL), which is a type of non-Hodgkin lymphoma. It is characterized by the presence of a mass in the mediastinum, the central compartment of the thoracic cavity. PMBCL primarily affects young adults, with a higher prevalence in females.

Pathophysiology[edit | edit source]

PMBCL originates from B-cells, a type of white blood cell that plays a crucial role in the immune response. These B-cells undergo malignant transformation and proliferate uncontrollably, forming a tumor in the mediastinum. The exact cause of this transformation is not fully understood, but it involves genetic mutations and alterations in signaling pathways that regulate cell growth and survival.

Clinical Presentation[edit | edit source]

Patients with PMBCL typically present with symptoms related to the mass effect of the tumor in the mediastinum. Common symptoms include:

Diagnosis[edit | edit source]

The diagnosis of PMBCL involves a combination of clinical evaluation, imaging studies, and histopathological examination.

Imaging[edit | edit source]

Imaging studies such as chest X-ray, computed tomography (CT) scan, and positron emission tomography (PET) scan are used to assess the size and extent of the mediastinal mass.

Histopathology[edit | edit source]

A biopsy of the mediastinal mass is essential for definitive diagnosis. Histopathological examination reveals large atypical B-cells with a diffuse growth pattern. Immunohistochemistry is used to confirm the B-cell origin of the tumor by demonstrating the expression of B-cell markers such as CD20.

Treatment[edit | edit source]

The treatment of PMBCL typically involves a combination of chemotherapy and radiation therapy.

Chemotherapy[edit | edit source]

The standard chemotherapy regimen for PMBCL is R-CHOP, which includes:

Radiation Therapy[edit | edit source]

Radiation therapy may be used in conjunction with chemotherapy to improve local control of the disease, especially in cases where there is residual disease after chemotherapy.

Prognosis[edit | edit source]

The prognosis for patients with PMBCL has improved significantly with modern treatment regimens. The overall survival rate is favorable, particularly for patients who achieve complete remission after initial therapy.

See also[edit | edit source]

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Contributors: Prab R. Tumpati, MD