A Rare Case of Primary Mediastinal B-Cell Lymphoma – The Great Masquerade

Abstract

Primary mediastinal B-cell lymphoma (PMBCL) is a relatively rare lymphoma subtype affecting mainly seen in young adults with female predominance. It constitutes approximately 2–4% of all non-Hodgkin’s lymphomas (NHLs). No risk factors for this type have been identified but it may be related to 5533 C>A mutation in the MLL gene. Its molecular signature and clinical features resemble classical Hodgkin’s lymphoma. PMBCL belongs to a group of aggressive diffuse large B-cell lymphomas. 2008 WHO classification distinguishes this lymphoma as a separate entity due to its specific clinical features and pathological features. Gene expression profile studies showed that it shares common features with classical Hodgkin’s lymphoma. The optimal chemotherapy for this lymphoma subtype has not been established. Furthermore, no convincing data are supporting the use of radiotherapy. Relatively low patient numbers are the main obstacle in conducting randomised prospective trials. Hence, therapeutic decisions have been based mainly on retrospective studies.