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The broad and challenging landscape of extranodal lymphoproliferations

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Abstract

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.

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Acknowledgements

The authors would like to acknowledge all workshop participants for submitting their case and for granting permission for the use of images in this review.

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All authors conceived and designed the review; all authors participated in the collection, discussion and the final diagnostic workup of the submitted cases. SD, MP, AT-G and GO wrote the first draft of the manuscript that was edited and reviewed by all other authors. All authors gave their final approval for publication.

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Correspondence to German Ott.

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Dotlic, S., Ponzoni, M., King, R.L. et al. The broad and challenging landscape of extranodal lymphoproliferations. Virchows Arch 476, 633–646 (2020). https://doi.org/10.1007/s00428-019-02702-w

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