"PMBCL accounts for approximately 5–6% of all large B-cell lymphomas and typically affects younger adults with a female preponderance 3:2, and median age in the 30s. (On the other hand, diffuse large B-cell lymphoma not otherwise specified comprises over 80% of cases and typically presents in older adults with a male predilection and disseminated nodal or extranodal disease) 5."
"pan–B-cell markers (CD20, CD79a, PAX5), CD30 positivity (usually moderate and heterogeneous), and CD23 positivity"
"PMBCL accounts for approximately 5–6% of all large B-cell lymphomas and typically affects younger adults with a female preponderance 3:2, and median age in the 30s. (On the other hand, diffuse large B-cell lymphoma not otherwise specified comprises over 80% of cases and typically presents in older adults with a male predilection and disseminated nodal or extranodal disease) 5."
"PMBCL almost always presents as a rapidly growing bulky mass in the anterior mediastinum, frequently with local invasion and compressive symptoms such as airway stenosis, cardiac tamponade or SVC syndrome (which is present in up to 35% of cases) 1,2."
"PMBCL almost always presents as a rapidly growing bulky mass in the anterior mediastinum, frequently with local invasion and compressive symptoms such as airway stenosis, cardiac tamponade or SVC syndrome (which is present in up to 35% of cases) 1,2."
"PMBCL is derived from thymic medullary B cells, similar to classic Hodgkin lymphoma, typically demonstrating 6,7:"
"negative or only weakly positive CD10, weak or absent surface and cytoplasmic immunoglobulin expression and variable PU.1.MAL protein expression"
"tumour cell negativity for CD15 and CD3, - helping to distinguish PMBCL from classic Hodgkin lymphoma and T-cell neoplasms"
"Genetically PMBCL has a unique molecular profile and immune evasion phenotype:"
"mutations in BCL6, PIM1 and other genes involved in JAK-STAT and NF-κB signalling pathways"
"mutations in BCL6, PIM1 and other genes involved in JAK-STAT and NF-κB signalling pathways"
"PMBCL presents as a large prevascular mediastinal mass. Middle and posterior mediastinal involvement suggests an alternative diagnois of conventional diffuse large B-cell lymphoma or T-cell lymphoma 1."
"SVC obstruction: up to 35% cases 1"
"Urgent treatment such as pericardial drainage and tracheal or bronchial stents may be necessary to avoid life-threatening complications. PMBCL is curable with immunochemotherapy regimens, most commonly EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, rituximab) or R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone), and this may be followed by mediastinal radiotherapy depending on response 8."
"Urgent treatment such as pericardial drainage and tracheal or bronchial stents may be necessary to avoid life-threatening complications. PMBCL is curable with immunochemotherapy regimens, most commonly EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, rituximab) or R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone), and this may be followed by mediastinal radiotherapy depending on response 8."
"Urgent treatment such as pericardial drainage and tracheal or bronchial stents may be necessary to avoid life-threatening complications. PMBCL is curable with immunochemotherapy regimens, most commonly EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, rituximab) or R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone), and this may be followed by mediastinal radiotherapy depending on response 8."
"pleural effusion and pericardial effusion are signs of aggressive tumour biology with increased risk of treatment failure, relapse and recurrence, and indicate a need for closer monitoring; hazard ratios for overall survival exceed 4 in patients treated with R-CHOP without radiotherapy"
"thymic carcinoma - more aggressive than PMBCL, with local invasion, tumour spread and metastases"
"negative or only weakly positive CD10, weak or absent surface and cytoplasmic immunoglobulin expression and variable PU.1.MAL protein expression"
"direct involvement of thoracic viscera (such as lung, pericardium or heart) and infra-diaphragmatic spread, which are markers of extranodal and extensive disease, both of which independently predict poor outcomes"
Expected headings
"Complications"
"Primary mediastinal large B-cell lymphoma is a unique entity, recognised in the WHO classification of lymphoma, with distinct biology, presentation and therapeutic implications compared to other large B-cell lymphomas."
"negative or only weakly positive CD10, weak or absent surface and cytoplasmic immunoglobulin expression and variable PU.1.MAL protein expression"
"mutations in BCL6, PIM1 and other genes involved in JAK-STAT and NF-κB signalling pathways"
"direct involvement of thoracic viscera (such as lung, pericardium or heart) and infra-diaphragmatic spread, which are markers of extranodal and extensive disease, both of which independently predict poor outcomes"
"pleural effusion and pericardial effusion are signs of aggressive tumour biology with increased risk of treatment failure, relapse and recurrence, and indicate a need for closer monitoring; hazard ratios for overall survival exceed 4 in patients treated with R-CHOP without radiotherapy"
"direct invasion of lung, pericardium and chest wall, and transdiaphragmatic spread"
"Urgent treatment such as pericardial drainage and tracheal or bronchial stents may be necessary to avoid life-threatening complications. PMBCL is curable with immunochemotherapy regimens, most commonly EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, rituximab) or R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone), and this may be followed by mediastinal radiotherapy depending on response 8."
"pleural effusion and pericardial effusion are signs of aggressive tumour biology with increased risk of treatment failure, relapse and recurrence, and indicate a need for closer monitoring; hazard ratios for overall survival exceed 4 in patients treated with R-CHOP without radiotherapy"
"mediastinal germ cell tumours, - more common in males; high-grade tumours are almost exclusively seen in males and have elevated serum markers"