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An unusual case of intracerebral Non-Langerhans cell histiocytosis with review of the literature

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Aims

In the face of an intracerebral case of Non-Langerhans cell histiocytosis (NLCH), the diagnostic procedure and the clinical handling of this rare histiocytic tumorous lesion are demonstrated and differential diagnoses as Rosai-Dorfman or Erdheim Chester diseases are discussed.

Methods

A stereotactic serial biopsy of frontal brain was taken from a patient with unclear contrast enhancing masses around the sellar region, the hypothalamus, and a wide lining intraventricular. Smear preparations, conventional histology, immunohistochemistry and electron microscopy were done to rule out the etiology of the tumorous lesion.

Results

The morphological analysis of our case revealed a histiocytic proliferation, which, in view of the immunohistochemical and ultrastructural findings, was classified as Non-Langerhans cell histiocytosis. No infectious agents could be detected and no lymphoma-like features could be observed.

Conclusion

This case shows the unusual manifestation of a primary intracerebral Non-Langerhans cell histiocytosis, which begins as a solid mass around the sellar region and shows a remarkable extension as a flat lining of inner and outer liquor spaces. Stereotactic brain biopsy technique today is a valuable high tech tool to clarify intracerebral tumorous lesions with a minimum of risk for the patient and with the possibility to perform all necessary tissue investigations.

Author information

Correspondence to K Mueller.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Keywords

  • Tumorous Lesion
  • Contrast Enhance
  • Sellar Region
  • Ultrastructural Finding
  • Unusual Manifestation