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Chapter 28 - Histiocytic Inflammatory Neoplasms/Lesions

from Section VI - Histiocytic Disorders and Neoplasms

Published online by Cambridge University Press:  25 January 2024

Xiayuan Liang
Affiliation:
Children’s Hospital of Colorado
Bradford Siegele
Affiliation:
Children’s Hospital of Colorado
Jennifer Picarsic
Affiliation:
Cincinnati Childrens Hospital Medicine Center
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Summary

Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), and Rosai-Dorfman-Destombes disease (RDD) can each manifest as a focal lesion, as multiple lesions, or as a widespread systemic disorder with organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process in children, with more frequent adult presentations. New distinct emerging entities covered include ALK-positive histiocytosis and post-leukemia/lymphoma histiocytic lesions. These histiocytic lesions are now best classified as myeloid-derived inflammatory neoplastic disorders composed of clonal dendritic- or macrophage-/monocyte-derived cells that infiltrate tissues and are driven by recurrent kinase-activating alterations, most often in the mitogen-activated protein kinase (MAPK), PI3K-AKT, and receptor tyrosine kinase (RTK) signaling pathways, which have all had a long history of being associated with human neoplasia, with ERK overexpression noted in many of these neoplasms (Fig. 28.1; Table 28.1) (1–3). Furthermore, these oncological signaling cascades are critical to the intranuclear regulation of transcription factors that serve as key factors influencing cellular proliferation, survival, and differentiation (4).

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Publisher: Cambridge University Press
Print publication year: 2024

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To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

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Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

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