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Mantle cell lymphoma—a spectrum from indolent to aggressive disease

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Abstract

Mantle cell lymphoma (MCL) is a distinctive lymphoma type generally characterized by the presence of CCND1 translocation and overexpression of cyclin D1. MCL usually presents with advanced stage and rapid clinical progression. The diagnosis is in most instances uncomplicated but cases with variant morphologies or immunophenotypes, especially cyclin D1-negative cases, may cause diagnostic difficulties. During the mantle cell lymphoma (MCL) session at the European Association of Haematopathology/Society for Hematopathology workshop 2014 held in Istanbul, Turkey, submitted cases illustrated interesting features such as unusual morphology or immunophenotypes. In several submitted cases of cyclin D1-positive MCL, CCND1 rearrangement could not be detected by t(11;14)(q13;q32) dual-color dual-fusion FISH but was suggested by CCND1 break-apart probes, and advantages and disadvantages of different FISH probes were highlighted. Three cyclin D1-negative MCL cases were submitted. These were identified by SOX11 immunohistochemistry and found to carry CCND2 translocations and/or to express high levels of cyclin D2 mRNA. Features associated with aggressive clinical course were presented including high expression of p53 protein and MYC aberrations. The need to integrate histological, immunophenotypic, genetic, and clinical data to arrive at the correct diagnosis was emphasized.

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Acknowledgments

We thank all participants of the workshop for submitting their cases. The permission to use their images in this review is also gratefully acknowledged. The authors thank Dr. Irina Bonzheim from the Institute of Pathology, University of Tübingen, Germany, for performing the RT-PCR for cyclin D1 and cyclin D2 mRNA in several of the cases and Dr Itziar Salaverria from Insitut d’Investigació Biomèdica Augusti Pi i Sunyer (IDIBAPS) of Barcelona for performing FISH studies for CCND2.

Conflict of interest

We declare that we have no competing interests.

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Authors and Affiliations

  1. Department of Laboratory Medicine, Division of Pathology, Karolinska Institutet and Karolinska University Hospital Huddinge, SE 141 86, Stockholm, Sweden

    Birgitta Sander

  2. Institute of Pathology and Neuropathology, Eberhard Karls University of Tübingen and Comprehensive Cancer Center, University Hospital Tübingen, Tübingen, Germany

    Leticia Quintanilla-Martinez

  3. Department of Clinical Pathology, Robert-Bosch-Hospital and Dr. Margarete Fischer-Bosch Institute of Clinical Pharmacology, Stuttgart, Germany

    German Ott

  4. Department of Bio-Pathology, Institut Paoli-Calmettes, Marseille, France

    Luc Xerri

  5. Department of Pathology, Ankara University School of Medicine, Sihhiye, Ankara, Turkey

    Isinsu Kuzu

  6. Queen Elizabeth Hospital, Hong Kong, SAR, China

    John K. C. Chan

  7. Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    Steven H. Swerdlow

  8. Hospital Clinic, University of Barcelona, Barcelona, Spain

    Elias Campo

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  1. Birgitta Sander
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Sander, B., Quintanilla-Martinez, L., Ott, G. et al. Mantle cell lymphoma—a spectrum from indolent to aggressive disease. Virchows Arch 468, 245–257 (2016). https://doi.org/10.1007/s00428-015-1840-6

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