Juvenile Xanthogranuloma and Related Non-LCH Disorders

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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.

Keywords

Juvenile xanthogranuloma Non-LCH disorders related to JXG Cutaneous JXG-like disorders 
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© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department Woman-Mother-Child, Divisions of Pediatric Oncology and HematologyUniversity Hospital of LausanneLausanneSwitzerland
  2. 2.Department of Pediatrics, Division of Hematology/OncologyHospital for Sick Children, University of TorontoTorontoCanada

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