Bile Duct Targeting or Preservation : Contrasting Liver Histology in Langerhans Cell Histiocytosis and Disseminated Juvenile Xanthogranuloma

Article Article scientifique Article Bile Duct Targeting or Preservation : Contrasting Liver Histology in Langerhans Cell Histiocytosis and Disseminated Juvenile Xanthogranuloma eng 2025-10-31 author Däniker Margaux Isabelle Faculty of Medicine, University of Geneva, Switzerland. 929165 author Baleydier Frédéric Unit of Paediatric Oncology and Haematology, Department of Women, Child and Adolescent, Geneva University Hospitals, CANSEARCH Research Platform in Paediatric Oncology, Switzerland. 987669 Département de Pédiatrie, Gynécologie et Obstétrique (PETRI) author Rock Nathalie Swiss Pediatric Liver Center, Department of Women, Child and Adolescent, Geneva University Hospitals, Switzerland.,Pediatric Gastroenterology, Hepatology and Nutrition Unit, Division of Pediatric Specialties, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, Switzerland. 826963 author Menzinger Sébastien Division of Clinical Pathology, Diagnostic Department, and Division of Dermatology and Venereology, Department of Medicine, Geneva University Hospitals, Switzerland. 633099 author Wildhaber Barbara Swiss Pediatric Liver Center, Department of Women, Child and Adolescent, Geneva University Hospitals, Switzerland.,Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, Switzerland. 595596 Département de Pédiatrie, Gynécologie et Obstétrique (PETRI) author Mclin Valérie Anne Swiss Pediatric Liver Center, Department of Women, Child and Adolescent, Geneva University Hospitals, Switzerland.,Pediatric Gastroenterology, Hepatology and Nutrition Unit, Division of Pediatric Specialties, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, Switzerland. 799651 Département de Pédiatrie, Gynécologie et Obstétrique (PETRI) author Rougemont-Pidoux Anne-Laure 0000-0002-1785-792X Swiss Pediatric Liver Center, Department of Women, Child and Adolescent, Geneva University Hospitals, Switzerland.,Pediatric and Fetoplacental Unit & Clinical Molecular Pathology Unit, Division of Clinical Pathology, Diagnostic Department, Geneva University Hospitals, Switzerland. 29140 Département de pathologie et immunologie (PATIM) 10.1177/10935266251385405 41170802 1093-5266 https://journals.sagepub.com/doi/10.1177/10935266251385405 Liver involvement by histiocytic and dendritic cell neoplasms signals high-risk disease, often necessitating closer monitoring and aggressive management. Severe cases may progress to liver failure, requiring transplantation. Liver involvement occurs in about one-third of patients with systemic juvenile xanthogranuloma (JXG) and 20% to 60% of pediatric patients with Langerhans cell histiocytosis (LCH), particularly in multiorgan disease. Tyrosine kinase inhibitors show promise in LCH treatment, but optimal timing for treatment cessation remains uncertain. We present 2 pediatric cases, 1 with LCH, and the other with disseminated JXG, along with a literature review emphasizing liver histopathology and transplant considerations. These cases highlight distinct histological patterns. In LCH, progressive bile duct destruction led to ductopenic cholestatic cirrhosis and secondary sclerosing cholangitis. In contrast, in the case of JXG, bile ducts remained intact despite being surrounded by histiocytes. In both, disease localization to larger, segmental portal tracts may reduce liver biopsy sensitivity. In LCH, BRAF inhibitor therapy triggered a granulomatous reaction that could mimic disease recurrence in the liver graft. Other histiocytoses typically spare the bile ducts and do not cause biliary cirrhosis. Recognizing these distinct infiltration patterns can aid diagnosis and management. BRAF CSF1R PTPN11 Granulomatous hepatitis Secondary sclerosing cholangitis Dewey 616.07 Dewey 616 Dewey 618 137 Département de pathologie et immunologie 21F 58 Département de pédiatrie, gynécologie et obstétrique 223 56 Département de médecine 221 false 559 Pathologies hépatiques congénitales et acquises de l'enfant 900 546 Recherche clinique en chirurgie pédiatrique 886 Pediatric and developmental pathology 97a237fc-4286-44c6-a5b1-f83d6e11e4a7 Article (Published version) Public Article.pdf 1033026 application/pdf CC BY-4.0 2686e78a8b3574d5a1ebe592eb5b7efe70b8c09c3a26696d059af265973e26f7 22b6fb49-576f-44fd-960a-9e5639f0bbcb Supplemental data Public sj-pptx-1-pdp-10.1177_1093526625138.pptx 62192 application/vnd.openxmlformats-officedocument.presentationml.presentation 7bc66d0f5e917c6f36a1d8e1f43775f91046e747b37d5cce2a47dbc0fe0606ee openAccess Faculté de médecine / Section de médecine fondamentale / Département de pathologie et immunologie Faculté de médecine / Section de médecine clinique / Département de pédiatrie, gynécologie et obstétrique Faculté de médecine / Section de médecine clinique / Département de médecine

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Bile Duct Targeting or Preservation : Contrasting Liver Histology in Langerhans Cell Histiocytosis and Disseminated Juvenile Xanthogranuloma	Pediatric and developmental pathology			2025	19	107

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Däniker, Margaux Isabelle