The broad and challenging landscape of extranodal lymphoproliferations

Virchows Arch. 2020 May;476(5):633-646. doi: 10.1007/s00428-019-02702-w. Epub 2019 Nov 22.


Two sessions in the workshop of the 19th meeting of the European Association for Haematopathology termed "challenging extranodal lymphoproliferations" and "extranodal non-site-specific lymphoproliferations", dealt with a series of heterogenous cases. These included lymphoproliferations of all cell lineages, from reactive lesions mimicking lymphomas through indolent lymphoid neoplasia and tumours with unclear biological behaviour to aggressive and transformed lymphomas. The themes addressed included cases with borderline features between hyperplastic and neoplastic lesions, the diagnostic spectrum of IgG4-related disease, T cell lymphoproliferations arising in extranodal sites with presumed indolent behaviour, diverse clinical presentations of intravascular large B cell lymphoma, diagnostic problems encountered with tumours displaying plasmablastic morphology, pitfalls concerning rare entities like adult T cell lymphoma/leukaemia (ATLL) and extranodal natural killer/T cell (NK/T) lymphomas, and unusual clinical presentations of various lymphomas. Altogether, within the frame of these two sessions, 75 cases remarkably differing in their clinical background, genetic features and overall need for a meticulous diagnostic approach were presented and discussed. In this paper, the salient points raised during the discussion of the cases, current diagnostic concepts and recommendations relevant to the diagnosis of these lymphoproliferations are described.

Keywords: Adult T cell lymphoma/leukaemia; Extranodal Hodgkin lymphoma; Extranodal NK/T cell lymphoma; High grade B cell lymphoma; IgG4-related disease; Indolent lymphoproliferations; Intravascular large B cell lymphoma; Plasmablastic lymphoma; Plasmablastic myeloma; Transformed and composite lymphomas.

Publication types

  • Review

MeSH terms

  • Education
  • Humans
  • Immunoglobulin G4-Related Disease / classification
  • Immunoglobulin G4-Related Disease / diagnosis*
  • Immunoglobulin G4-Related Disease / genetics
  • Immunoglobulin G4-Related Disease / pathology
  • Lymphoma, B-Cell / classification
  • Lymphoma, B-Cell / diagnosis*
  • Lymphoma, B-Cell / genetics
  • Lymphoma, B-Cell / pathology
  • Lymphoma, T-Cell / classification
  • Lymphoma, T-Cell / diagnosis*
  • Lymphoma, T-Cell / genetics
  • Lymphoma, T-Cell / pathology
  • Lymphoproliferative Disorders / classification
  • Lymphoproliferative Disorders / diagnosis*
  • Lymphoproliferative Disorders / genetics
  • Lymphoproliferative Disorders / pathology