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Paediatric neuroradiological aspects of Langerhans cell histiocytosis

  • Paediatric Neuroradiology
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Abstract

Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic–pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse.

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We declare that we have no conflict of interest.

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Authors and Affiliations

  1. Department of Radiology , University Hospital K.U.Leuven, Herestraat 49, 3000, Leuven, Belgium

    Philippe Demaerel

  2. Paediatric Haemato-oncology/Neuro-oncology, University Hospital K.U.Leuven, Leuven, Belgium

    Stefaan Van Gool

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Correspondence to Philippe Demaerel.

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Demaerel, P., Van Gool, S. Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology 50, 85–92 (2008). https://doi.org/10.1007/s00234-007-0323-0

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  • DOI: https://doi.org/10.1007/s00234-007-0323-0

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