IV. Masses in the mediastinum: primary mediastinal lymphoma and intermediate types.

نویسنده

  • Peter W M Johnson
چکیده

Lymphoma in the mediastinum is the subject of active research and controversy. Patients are usually young and often present with acute compressive symptoms; the pathology can be difficult to interpret; our understanding of the biology is in evolution and the approach to treatment is also changing, in particular the types of induction immunochemotherapy that are preferred and the role of consolidation radiotherapy. Recent data on functional imaging has given useful results, and important prospective trials are underway which will help with future decision making. Primary mediastinal B-cell lymphoma (PMBL) was recognized clinically as a particular entity 25 years ago and is now known to be distinct from other types of diffuse large B-cell lymphoma (DLBL) in its epidemiology, biology and clinical behaviour [1]. In common with nodular sclerosing Hodgkin lymphoma in the mediastinum, it is thought to originate from thymic B-cells [2], and there is an intermediate entity which lies between these two types, currently termed mediastinal gray-zone lymphoma (MGZL) [3]. PMBL is relatively uncommon, comprising around 3% of all NHL, and up to 10% of DLBL. It is found worldwide, is more common in females and most often presents in young adults, with a median age of 35 [4]. MGZL is even less common, but appears to have similar clinical characteristics. Bothmay present with local compressive symptoms including cough, dysphagia, hoarseness and oedema from superior vena cava compromise. Because of these, presentation is usually with early stage but bulky disease, and extrathoracic spread is rare at diagnosis. Around two thirds of patients have elevated lactate dehydrogenase levels at diagnosis [5,6].

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عنوان ژورنال:
  • Hematological oncology

دوره 33 Suppl 1  شماره 

صفحات  -

تاریخ انتشار 2015