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Lymphomas arising in immune-privileged sites: insights into biology, diagnosis, and pathogenesis

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Abstract

Session 2 of the 2018 European Association of Hematopathology/Society for Hematopathology Workshop focused on lymphomas arising in immune-privileged sites: both lymphomas arising in the traditionally described “immune sanctuary” sites of the central nervous system (CNS) and testes, as well as those arising at sites of local immune privilege. Primary CNS large B cell lymphoma and primary testicular large B cell lymphoma were discussed, and the biology of these unique tumors was highlighted by several cases showing the classic mutation profile including MYD88 L265P and CD79B. The tendency of these tumors to involve both the CNS and testis was also reinforced by several cases. Four cases of low-grade B cell lymphomas (LGBCL) of the CNS were discussed. Two were classic Bing-Neel syndrome associated with LPL, and two were LGBCL with plasmacytic differentiation and amyloid deposition without systemic disease. Rare examples of systemic T and NK cell lymphomas involving the CNS were also discussed. Several cases of breast implant–associated anaplastic large cell lymphoma (BI-ALCL) were submitted showing the typical clinicopathologic features. These cases were discussed along with a case with analogous features arising in a patient with a gastric band implant, as well as large B cell lymphomas arising alongside foreign materials. Finally, large B cell lymphomas arising in effusions or localized sites of chronic inflammation (fibrin-associated diffuse large B cell lymphoma [DLBCL] and DLBCL associated with chronic inflammation) were described. The pathogenesis of all of these lymphomas is believed to be related to decreased immune surveillance, either innate to the physiology of the organ or acquired at a local site.

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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.

Author information

Authors and Affiliations

  1. Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

    Rebecca L. King

  2. Department of Pathology, Queen Elizabeth University Hospital, Glasgow, UK

    John R. Goodlad

  3. Department of Cellular Pathology, SIHMDS, Barts Health NHS Trust and Centre for Haemato-Oncology, Barts Cancer Institute, London, UK

    Maria Calaminici

  4. Department of Pathology and Cytology, University Hospital Centre Zagreb, University of Zagreb Medical School, Zagreb, Croatia

    Snjezana Dotlic

  5. Anatomic Pathology Service and Translational Hematopathology Lab, Hospital Universitario Marques de Valdecilla/IDIVAL, Santander, Spain

    Santiago Montes-Moreno

  6. Department of Pathology, Hematopathology Section and Lymphnode Registry, University Hospitals Schleswig-Holstein, Christian-Albrecht-University, Kiel, Germany

    Ilske Oschlies

  7. Ateneo Vita-Salute San Raffaele and Unit of Lymphoid Malignancies, San Raffaele Scientific Institute, Milan, Italy

    Maurilio Ponzoni

  8. Department of Hematopathology, Hospices Civils de Lyon/Université Lyon 1, Lyon, France

    Alexandra Traverse-Glehen

  9. Department of Clinical Pathology, Robert-Bosch-Krankenhaus, Stuttgart, Germany

    German Ott

  10. Department of Pathology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA

    Judith A. Ferry

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Contributions

All authors conceived and designed the review; all authors participated in the collection, discussion, and the final diagnostic workup of the submitted cases. RLK and JAF 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 Judith A. Ferry.

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King, R.L., Goodlad, J.R., Calaminici, M. et al. Lymphomas arising in immune-privileged sites: insights into biology, diagnosis, and pathogenesis. Virchows Arch 476, 647–665 (2020). https://doi.org/10.1007/s00428-019-02698-3

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  • DOI: https://doi.org/10.1007/s00428-019-02698-3

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