Abstract
Histiocytosis is a heterogeneous group of disease entities, comprised by two main categories, namely Langerhans and non-Langerhans cell histiocytoses. Central nervous system involvement in histiocytosis is considered very rare and is often secondary to affection of anatomically related bone structures and/or multi-organ disease. We present a never-before described case of rare childhood histiocytosis with hybrid features of Langerhans cell histiocytosis and juvenile xanthogranuloma confined to the central nervous system in a 2- and a half-year-old boy with distinct treatment response to clofarabine. The case also emphasizes the diagnostic significance of stereotactic brain biopsy.
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Acknowledgements
We would like to acknowledge Dr. Jennifer Picarsic, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, for her important contribution to the pathological examination and diagnosis.
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Foss-Skiftesvik, J., Scheie, D., Klausen, C. et al. Rare childhood hybrid histiocytosis of the central nervous system—diagnosed by stereotactic brain biopsy with marked treatment response to clofarabine. Childs Nerv Syst 34, 2321–2324 (2018). https://doi.org/10.1007/s00381-018-3859-x
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DOI: https://doi.org/10.1007/s00381-018-3859-x