gms | German Medical Science

68th Annual Meeting of the German Society of Neurosurgery (DGNC)
7th Joint Meeting with the British Neurosurgical Society (SBNS)

German Society of Neurosurgery (DGNC)

14 - 17 May 2017, Magdeburg

Article

Send article

Isolated Intracerebral Langerhans Cell Histiocytosis with Multifocal Lesions

Meeting Abstract

Search Medline for

  • Julia Velz - University Hospital Zürich, Department of Neurosurgery, Zürich, Switzerland
  • Marian Christoph Neidert - University Hospital Zurich, Department of Neurosurgery, Zürich, Switzerland
  • Elisabeth Rushing - University Hospital Zürich, Department of Neuropathology, Zürich, Switzerland
  • Oliver Bozinov - Klinik für Neurochirurgie, Universitätsspital Zürich, Zürich, Switzerland

Deutsche Gesellschaft für Neurochirurgie. Society of British Neurological Surgeons. 68. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), 7. Joint Meeting mit der Society of British Neurological Surgeons (SBNS). Magdeburg, 14.-17.05.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. DocP 188

doi: 10.3205/17dgnc751, urn:nbn:de:0183-17dgnc7516

Published: June 9, 2017

© 2017 Velz et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Objective: Langerhans cell histiocytosis (LCH) is a rare and extremely heterogeneous disease with a largely unknown etiology and an unpredictable clinical course. Most cases of LCH occur in children. The clinical picture in LCH can vary from single organ involvement, usually of either bone or skin, to multiple organ involvement. However, central nervous system (CNS) involvement is rare. When involving the CNS, abnormalities of the hypothalamic-pituitary region are common. Here, we report the case of a 7-year-old boy with isolated intracerebral LCH with multifocal lesions.

Methods: Clinical investigation, cranial magnetic resonance imaging, CSF examination, as well as a FDG PET of the entire body was performed. A mini-craniotomy was performed to resect the frontal lesion and histological examination of the brain tissue was done. Hematoxylin-eosin stain, Immunohistochemical studies with langerin (CD207), S100 protein and CD 68, as well as PCR were performed.

Results: MRI findings revealed not only a space-occupying intra- and suprasellar lesion, but also multiple lesions in the brain stem, supratentorial white matter and the meninges. Histopathological examination revealed features of intracerebral LCH.

Conclusion: The clinical and radiological heterogeneity of cerebral LHC suggests a diversity of differential diagnoses ranging from inflammatory diseases such as neurosarcoidosis, infections such as neurocysticercosis to neoplasms such as germinoma. Thus surgery is highly recommended to obtain tissue sample.