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Indolent lymphomas in the pediatric population: follicular lymphoma, IRF4/MUM1+ lymphoma, nodal marginal zone lymphoma and chronic lymphocytic leukemia

Abstract

Indolent lymphomas in the pediatric population were discussed during the 2014 European Association for Haematopathology/Society of Hematopathology workshop in Istanbul, Turkey. This session was focused on pediatric-type follicular lymphoma (FL), and its differential diagnosis with the newly recognized entity of IRF4/MUM1+ lymphomas mainly involving Waldeyer’s ring. The differential diagnosis between t(14;18) negative FL grade 1/2 and pediatric-type FL in adults was highlighted. The overlapping pathological and clinical features between FL and nodal marginal zone lymphoma (NMZL) in children and young adults were recognized and morphologic and immunophenotypical criteria helpful for the differential diagnosis were presented. Both pediatric-type FL and NMZL are indolent processes that should be distinguished from atypical lymphoid hyperplasia of the tonsils and lymph nodes. The demonstration of a B cell monoclonal population by molecular studies is strongly recommended for the diagnosis. Recognition of these indolent variants to avoid overtreatment was emphasized. Whereas most indolent lymphomas in the pediatric population show characteristic clinical, pathologic, and genetic features that differ from the adult counterpart, other rare indolent lymphoid tumors such as chronic lymphocytic leukemia (CLL) have similar characteristics. In this report, novel findings, areas of special interest, and diagnostic challenges emerging from the cases submitted to the workshop will be discussed.

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Acknowledgments

The authors thank Dr. Irina Bonzheim and Janine Schmidt from the Institute of Pathology, University of Tübingen, Germany, for performing the molecular analysis of B-cell clonality and MYD88 L265P mutation in cases submitted to the workshop. The authors also thank Dr. Reiner Siebert from the Institute of Human Genetics, University, Campus Kiel, Germany, for performing the fluorescence in situ hybridization analysis using an IRF4/MUM1 break-apart probe in two cases. The authors also thank the workshop participants for their case submissions and for use of their images in this review.

Conflict of interest

The authors declare that they have no competing interests.

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Correspondence to Leticia Quintanilla-Martinez.

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Quintanilla-Martinez, L., Sander, B., Chan, J.K.C. et al. Indolent lymphomas in the pediatric population: follicular lymphoma, IRF4/MUM1+ lymphoma, nodal marginal zone lymphoma and chronic lymphocytic leukemia. Virchows Arch 468, 141–157 (2016). https://doi.org/10.1007/s00428-015-1855-z

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Keywords

  • Indolent pediatric lymphomas
  • Pediatric-type follicular lymphoma
  • Pediatric nodal marginal zone lymphoma
  • IRF4/MUM1+ lymphomas
  • Waldeyer’s ring lymphomas