The mediastinal large B-cell lymphoma is described as a distinct entity among high-grade non-Hodgkin’s lymphomas. A total of 340 cases representing most likely this particular lymphoma have been described throughout the literature during the last decade. The tumor is histologically characterized by a diffuse, large, clear cell proliferation, sometimes with sclerosis. Several immunophenotypic and genotypic studies have demonstrated the B-cell origin of this rare lymphoma. Its clinical behavior is aggressive with the tendency to invade adjacent structures like a solid tumor. Unusual extranodal sites are reported to be involved quite often in the disease, while enlargement of peripheral lymph nodes is found rarely. Additional clinical characteristics are prevalence of affected females over males and a young median age of the patients. Radiotherapy alone as well as chemotherapy with CHOP reveal poor remission rates. However, multicomponent regimens like MACOP-B, COP-BLAM/IMVP-16 or high-dose MTX-containing chemotherapy provide complete remission rates in 60-90% of the patients. In summary, mediastinal large B-cell lymphoma is a clinicopathologic entity that must be distinguished from other primary mediastinal tumors. When treated with intensive multicomponent chemotherapy, there is a potential of cure in a substantial number of patients.

Keywords:
Mediastinal large B-cell lymphoma, Histomorphology, Immunophenotype, Genotype, Treatment of mediastinal large B-cell lymphoma
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© 1994 S. Karger AG, Basel
1994
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