Primary mediastinal large B cell lymphoma
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jon C Aster, MD
Jon C Aster, MD
- Professor of Pathology
- Harvard Medical School
- Jonathan W Friedberg, MD
Jonathan W Friedberg, MD
- Professor of Medicine
- James P Wilmot Cancer Center, University of Rochester
Primary mediastinal large B cell lymphoma (PMBL) is a clinically aggressive B cell lymphoma that is thought to arise from thymic (medullary) B cells. It has clinicopathologic features that are distinct from diffuse large B cell lymphoma (DLBCL) and shares some clinical and biologic features with classical nodular sclerosing Hodgkin lymphoma (HL).
This topic will discuss the clinical presentation, pathologic features, diagnosis, and treatment of PMBL. The diagnosis and treatment of systemic DLBCL and HL are presented separately. (See "Epidemiology, clinical manifestations, pathologic features, and diagnosis of diffuse large B cell lymphoma" and "Initial treatment of limited stage diffuse large B cell lymphoma" and "Overview of the treatment of classical Hodgkin lymphoma in adults".)
Primary mediastinal large B cell lymphoma (PMBL) comprises 7 percent of diffuse large B cell lymphomas (DLBCLs) (2.4 percent of all non-Hodgkin lymphomas) [1,2]. There is a female predominance and a median age at diagnosis in the third to fourth decade .
Oncologic emergencies — Patients present with a locally invasive anterior mediastinal mass originating in the thymus, with frequent airway compromise and superior vena cava (SVC) syndrome (image 1 and image 2) . In one report of 30 patients, for example, SVC syndrome was present in 57 percent at presentation . In addition, other patients without clinical SVC obstruction had evidence of compression of this vessel by computed tomography (CT) scan; in total, 80 percent had some evidence of SVC compromise. (See "Malignancy-related superior vena cava syndrome" and "Pathology of mediastinal tumors".)
Prompt recognition and management of SVC syndrome is critical. The clinical diagnosis of SVC syndrome is made on the basis of characteristic signs and symptoms of central venous obstruction. Dyspnea is the most common symptom. In addition, patients frequently complain of facial swelling or head fullness, which may be exacerbated by bending forward or lying down. Other symptoms include arm swelling, cough, chest pain, or dysphagia. Patients with cerebral edema may have headaches, confusion, or possibly coma. The diagnosis and management of malignancy-related SVC syndrome is presented separately. (See "Malignancy-related superior vena cava syndrome", section on 'Clinical manifestations and diagnosis'.)
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- CLINICAL PRESENTATION
- Oncologic emergencies
- Other signs and symptoms
- Radiographic features
- PATHOLOGIC FEATURES
- Genetic features
- Obtaining tissue
- Further evaluation
- Making the diagnosis
- DIFFERENTIAL DIAGNOSIS
- Systemic lymphoma with secondary mediastinal involvement
- Hodgkin lymphoma
- Pretreatment evaluation
- Initial therapy
- - Choice of therapy
- - Chemoimmunotherapy
- - Is radiation necessary?
- Evaluation of response
- Relapsed or refractory disease
- Clinical trials