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Juvenile Xanthogranuloma and Related Non-LCH Disorders

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Histiocytic Disorders

Abstract

JXG is a non-Langerhans cell histiocytosis (non-LCH) characterized by xanthomatous skin lesions that usually resolve spontaneously. In a minority of cases, systemic disease can occur and can be life threatening. Recent whole-exome sequencing of JXG cases did not show the BRAF-V600E mutation, although germline mutations in NF1 and NF2 and PI3KCD mutation have been reported in few patients. The standard treatment for solitary and symptomatic CNS JXG is surgical resection, while patients with multifocal cranial or systemic JXG have been successfully treated with LCH-like therapies such as vinblastine/prednisone or nucleoside analogues. Other JXG-like non-LCH disorders, such as cutaneous JXG-like disorders, xanthoma disseminatum, multicentric reticulohistiocytosis, and solitary reticulohistiocytoma, will also be discussed in this chapter.

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Authors and Affiliations

  1. Department Woman-Mother-Child, Divisions of Pediatric Oncology and Hematology, University Hospital of Lausanne, Lausanne, Switzerland

    Francesco Ceppi MD

  2. Department of Pediatrics, Division of Hematology/Oncology, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Oussama Abla MD

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  1. Francesco Ceppi MD
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Correspondence to Francesco Ceppi MD .

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  1. University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

    Oussama Abla

  2. Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Hamburg, Germany

    Gritta Janka

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Ceppi, F., Abla, O. (2018). Juvenile Xanthogranuloma and Related Non-LCH Disorders. In: Abla, O., Janka, G. (eds) Histiocytic Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-59632-7_17

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