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Histiocytic and dendritic cell neoplasms: what have we learnt by studying 67 cases

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Abstract

Tumors derived from histiocytic and dendritic cells encompass a large and heterogeneous group of neoplastic and reactive conditions, and their diagnosis is challenging both for pathologists and clinicians. Diagnosis is based on morphological and phenotypical findings, but hybrid features are not uncommon. Furthermore, recent studies uncovered the molecular mechanisms driving some of these tumors, improving diagnostic adequacy, and providing the basis for effective therapeutic breakthroughs.

Sixty-seven cases were submitted to the accessory cell and histiocytic neoplasms session at the European Association of Haematopathology/Society for Hematopathology workshop 2016 held in Basel, Switzerland. The cases included histiocytic sarcomas (HS), Langerhans cell tumors (LCT), Erdheim-Chester disease, interdigitating dendritic cell sarcomas (IDCS), indeterminate dendritic cell tumors (IND-DCT), follicular dendritic cell sarcomas, and blastic plasmacytoid dendritic cell neoplasms. Rosai-Dorfman disease and, more rare, conditions such as ALK-positive histiocytosis were also submitted. These cases illustrated classical and unexpected features at morphological, phenotypical, and molecular levels, providing a valuable compendium for pathologists confronting with these tumors.

The paper summarizes the most notable features of every single group of diseases, with comments about the most challenging issues, in the attempt to provide practical indications for their recognition.

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Acknowledgements

The study was supported by Associazione Italiana per la Ricerca sul Cancro (AIRC) grants 5x1000 10007 and IG 15762 (to S.A.P.) and by Fondazione Golgi, Brescia (to F.F.).

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Author notes

  1. Fabio Facchetti and Stefano Aldo Pileri have equally contributed to this study.

Authors and Affiliations

  1. Section of Pathology, Department of Molecular and Translational Medicine, University of Brescia, Spedali Civili, 25123, Brescia, Italy

    Fabio Facchetti & Luisa Lorenzi

  2. Unit of Hematopathology, European Institute of Oncology, 20141, Milan, Italy

    Stefano Aldo Pileri & Valentina Tabanelli

  3. Bologna University School of Medicine, Bologna, Italy

    Stefano Aldo Pileri

  4. Department of Laboratory Medicine and Pathology, The Mayo Clinic, Scottsdale, AZ, USA

    Lisa Rimsza

  5. Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA

    Stefania Pittaluga

  6. Institute of Pathology and Genetics, University Hospital Basel, Basel, Switzerland

    Stephan Dirnhofer

  7. Department of Pathology, Hopital Henri Mondor, INSERM U955, Université Paris-Est, Creteil, France

    Christiane Copie-Bergman

  8. Institute of Pathology, Lausanne University Hospital, Lausanne, Switzerland

    Laurence de Leval

  9. Institute of Pathology, University of Würzburg and Comprehensive Cancer Center Mainfranken, Würzburg, Germany

    Andreas Rosenwald

  10. Department of Histopathology, Royal Marsden Hospital, London and Sutton, UK

    Andrew Wotherspoon

  11. Institute of Pathology and Neuropathology and Comprehensive Cancer Center, University Hospital Tübingen, Tübingen, Germany

    Falko Fend

Authors
  1. Fabio Facchetti
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  2. Stefano Aldo Pileri
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  3. Luisa Lorenzi
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  4. Valentina Tabanelli
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  8. Christiane Copie-Bergman
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  9. Laurence de Leval
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Corresponding authors

Correspondence to Fabio Facchetti or Stefano Aldo Pileri.

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The authors declare that they have no conflicts of interest.

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Informed consent in this study is not applicable, since all cases were provided by different participants to the workshop and all data were completely anonymized.

Additional information

This article is part of the Topical Collection on Aggressive B-cell lymphomas and histiocytic neoplasias

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Facchetti, F., Pileri, S.A., Lorenzi, L. et al. Histiocytic and dendritic cell neoplasms: what have we learnt by studying 67 cases. Virchows Arch 471, 467–489 (2017). https://doi.org/10.1007/s00428-017-2176-1

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  • DOI: https://doi.org/10.1007/s00428-017-2176-1

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