Rare childhood hybrid histiocytosis of the central nervous system—diagnosed by stereotactic brain biopsy with marked treatment response to clofarabine

Child's Nervous System, Springer Nature, ISSN 0256-7040

Volume 34, 11, 2018

DOI:10.1007/s00381-018-3859-x, Dimensions: pub.1104410998, PMID: 29872899,


* Corresponding author



  1. (1) Rigshospitalet, grid.475435.4, Capital Region






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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