Primary mediastinal large B-cell lymphoma

Maurizio Martelli, Andrés J M Ferreri, Peter Johnson

Research output: Contribution to journalArticlepeer-review

Abstract

Primary mediastinal large B-cell lymphoma (PMLBCL) is a unique type of B-cell lymphoma probably arising from a putative thymic medulla B-cell. It constitutes 6-10% of all diffuse large B-cell lymphomas (DLBCL), occurring more often in young females. PMLBCL is characterized by a diffuse proliferation of medium to large B-cells associated with sclerosis and a degree of compartmentalisation. Its main molecular characteristics include: gains in 9p segments, p53 mutations, BCL-2 and MAL gene over-expression, somatic mutations of IgVH genes, BCL-6, PIM-1, PAX-5, RhoH/TTF, and c-MYC, and constitutional NF-κB activation. The gene expression signature of PMLBCL seems to be much closer to classic Hodgkin lymphoma than to DLBCL. PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome. Most PMLBCL patients have stage I-II, bulky disease, with pleural or pericardial effusions in a third of cases. Systemic symptoms, mainly fever or weight loss, are present in

Original languageEnglish
Pages (from-to)256-263
Number of pages8
JournalCritical Reviews in Oncology/Hematology
Volume68
Issue number3
DOIs
Publication statusPublished - Dec 2008

Keywords

  • Diffuse large B-cell lymphoma
  • Extranodal lymphomas
  • Mediastinum
  • Thymic lymphoma

ASJC Scopus subject areas

  • Oncology
  • Hematology
  • Geriatrics and Gerontology

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