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

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.

• Authors: J Foss-Skiftesvik, D Scheie, C Klausen, A Sehested, J Skjøth-Rasmussen
• Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (Childs Nerv Syst) Vol. 34 Issue 11 Pg. 2321-2324 (11 2018) ISSN: 1433-0350 [Electronic] Germany
• PMID: 29872899 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antimetabolites, Antineoplastic
• Clofarabine

Topics

• Antimetabolites, Antineoplastic (therapeutic use)
• Biopsy
• Brain (pathology)
• Central Nervous System Diseases (diagnosis, drug therapy, pathology)
• Child, Preschool
• Clofarabine (therapeutic use)
• Histiocytosis (diagnosis, drug therapy, pathology)
• Humans
• Male
• Stereotaxic Techniques