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Histiocytic and Dendritic Cell Neoplasms

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Practical Oncologic Molecular Pathology

Part of the book series: Practical Anatomic Pathology ((PAP))

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Abstract

Histiocytic and dendritic cell neoplasms are rare and represent a heterogeneous group of mature histiocytic and dendritic cell proliferations with unique clinicopathologic characteristics. Recurrent molecular genetic findings have greatly facilitated the diagnosis, classification, and treatment of histiocytic and dendritic cell neoplasms; particularly, mutations in the RAS-RAF-MEK-ERK pathway have been detected in most cases of Langerhans cell histiocytosis and Erdheim-Chester disease. Histiocytic and dendritic cell neoplasms may arise de novo or in association with B-cell, T-cell, or myeloid neoplasms, with the exception of follicular dendritic cell sarcoma. The transdifferentiated tumors retain the same IGH or TRG rearrangements and chromosomal changes as the lymphoid or myeloid neoplasms, although the morphologic and immunophenotypic features are usually different. In general, histiocytic and dendritic cell neoplasms show broad clinical, morphologic, immunophenotypic, and molecular genetic features, which may make an accurate diagnosis difficult. Therefore, it is critical to understand the clinicopathologic characteristics and molecular genetic features of each entity.

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Yale University School of Medicine, New Haven, CT, USA

    Nathan Paulson & Xi Wang

  2. Department of Pathology, Yale University School of Medicine, New Haven, CT, USA

    Zenggang Pan

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  1. Nathan Paulson
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  2. Xi Wang
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Correspondence to Zenggang Pan .

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Editors and Affiliations

  1. Department of Laboratory Medicine, Geisinger Health, Danville, PA, USA

    Yi Ding

  2. Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA

    Linsheng Zhang

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Paulson, N., Wang, X., Pan, Z. (2021). Histiocytic and Dendritic Cell Neoplasms. In: Ding, Y., Zhang, L. (eds) Practical Oncologic Molecular Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-73227-1_19

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  • DOI: https://doi.org/10.1007/978-3-030-73227-1_19

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