Clin Pediatr Hematol Oncol.
2024 Oct;31(2):47-51. 10.15264/cpho.2024.31.2.47.
Successful Treatment of Relapsed Disseminated Juvenile Xanthogranuloma with Central Nervous System Involvement
- Affiliations
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- 1Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children’s Hospital, Seoul, Korea
- 2Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
- KMID: 2560724
- DOI: http://doi.org/10.15264/cpho.2024.31.2.47
Abstract
- Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions. Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.
