Child's Nervous System

, Volume 34, Issue 11, pp 2321–2324 | Cite as

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

  • J. Foss-SkiftesvikEmail author
  • D. Scheie
  • C. Klausen
  • A. Sehested
  • J. Skjøth-Rasmussen
Case Report


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.


Histiocytosis Langerhans cell histiocytosis (LCH) Juvenile xanthogranuloma (JXG) Histiocytosis Central nervous system (CNS) Clofarabine 



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.

Compliance with ethical standards

Conflict of interest

On behalf of all authors, the corresponding author states that there are no conflicts of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurosurgeryRigshospitalet University HospitalCopenhagenDenmark
  2. 2.Department of NeuropathologyRigshospitalet University HospitalCopenhagenDenmark
  3. 3.Department of NeuroradiologyRigshospitalet University HospitalCopenhagenDenmark
  4. 4.Department of Paediatric OncologyRigshospitalet University HospitalCopenhagenDenmark

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