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

Rare childhood hybrid histiocytosis of the central nervous system—diagnosed by stereotactic... 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. . . . . Keywords Histiocytosis Langerhans cell histiocytosis (LCH) Juvenile xanthogranuloma (JXG) Histiocytosis Central nervous system (CNS) Clofarabine Introduction changes in the cerebellum and basal ganglia [4]. The patho- logical large mononuclear Langerhans cells are derived from Histiocytosis is a heterogeneous group of rare disease entities myeloid dendritic cells and typically express CD1a, S100, characterized by tissue invasion and accumulation of abnor- and Langerin [5]. Other pathological features include the mal histiocytic cells [1]. While one of the more common presence of CD8-positive T cells, demyelination, and axonal dendritic cell disorders, Langerhans cell histiocytosis (LCH) damage [6]. is still rare with an incidence rate http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Child's Nervous System Springer Journals

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

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Publisher
Springer Berlin Heidelberg
Copyright
Copyright © 2018 by Springer-Verlag GmbH Germany, part of Springer Nature
Subject
Medicine & Public Health; Neurosurgery; Neurosciences
ISSN
0256-7040
eISSN
1433-0350
D.O.I.
10.1007/s00381-018-3859-x
Publisher site
See Article on Publisher Site

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. . . . . Keywords Histiocytosis Langerhans cell histiocytosis (LCH) Juvenile xanthogranuloma (JXG) Histiocytosis Central nervous system (CNS) Clofarabine Introduction changes in the cerebellum and basal ganglia [4]. The patho- logical large mononuclear Langerhans cells are derived from Histiocytosis is a heterogeneous group of rare disease entities myeloid dendritic cells and typically express CD1a, S100, characterized by tissue invasion and accumulation of abnor- and Langerin [5]. Other pathological features include the mal histiocytic cells [1]. While one of the more common presence of CD8-positive T cells, demyelination, and axonal dendritic cell disorders, Langerhans cell histiocytosis (LCH) damage [6]. is still rare with an incidence rate

Journal

Child's Nervous SystemSpringer Journals

Published: Jun 5, 2018

References

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