Primary mediastinal large B cell lymphoma

Abstract

Primary mediastinal large B cell lymphoma (PMBCL) is an aggressive large B cell lymphoma originating in the mediastinum, that mainly expresses B cell surface molecules, such as CD19, CD20, CD22, andCD79a. Clinically, they are characterized by rapidly increasing anterior mediastinal masses, which can cause compression of the surrounding tissues. The diagnosis of PMBCL mainly depends on the pathological features, imaging examination and clinical features. Currently, the most commonly used therapeutic regimens are R-CHOP and R-EPOCH. Radiotherapy is beneficial in some patients, but it can also lead to long-term toxicity. The research and development of novel therapies are ongoing, and some studies have achieved encouraging results, including those conducted on chimeric antigen receptor-modified T (CAR-T) cell therapy and anti-PD-1 drugs. However, randomized controlled trials with larger sample sizes are still needed. Positron emission tomography-computed tomography (PET-CT) is mainly used to assess the curative effect after treatment and to guide the subsequent treatment strategy.

Large retrosternal non-hodgkin’s lymphoma presenting with recurrent syncope episodes and chest asymmetry: A case report

“…Even though our patient is a middle-aged woman, Mediastinal NHL usually occurs in young people [11]. Currently, our patient is being managed with R-CHOP regimen as it is considered the first-line treatment for the large B-cell non-Hodgkin's lymphoma according to the National Comprehensive Cancer Network (NCCN) guidelines [12].…”

Approach to Imaging of Mediastinal Masses

“…Lymphoma is the most common mediastinal tumor, accounting for about 50% to 60% of all mediastinal malignancies and can be Hodgkin or non-Hodgkin lymphoma [2,15,34]. Lymphomas arising primarily in the mediastinum are rare and comprise approximately 1% of all lymphomas [35]. Diagnosis of primary mediastinal lymphoma requires involvement of mediastinal lymph nodes, the thymus or both without involvement of any other lymph nodes or organs outside the mediastinum at the time of presentation.…”

Primary Mediastinal B-Cell Lymphoma Presenting as Cardiac Tamponade

“…Primary mediastinal B-cell lymphoma (PMBCL) is a rare subtype of non-Hodgkin lymphoma, only occurring in about 2-3% of patients diagnosed with non-Hodgkin lymphoma [ 1 , 2 ]. The tumor arises from a dysregulation in the Janus kinase signal transducer and activator of transcription (JAK-STAT), dysregulation of nuclear factor-κB (NF-κB) pathways, and amplification of 9p24.1 region of chromosome 9 [ 3 ].…”

“…Symptoms are typically due to the rapidly enlarging mass in the mediastinum, causing a mass effect on adjacent structures in the mediastinum including, but not limited to, the lungs and superior vena cava. The symptoms can also be due to pleural or pericardial effusions, which are rare complications of this malignancy [ 2 ]. Most cases occur in the anterior superior mediastinum in the regions of the thymus and are typically seen in females at approximately a median age of 35 years [ 2 , 3 ].…”

“…The symptoms can also be due to pleural or pericardial effusions, which are rare complications of this malignancy [ 2 ]. Most cases occur in the anterior superior mediastinum in the regions of the thymus and are typically seen in females at approximately a median age of 35 years [ 2 , 3 ]. As such, a diagnosis of PMBCL in males is uncommon.…”