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

  • K Mueller1,

    Affiliated with

    • M Trippel2,

      Affiliated with

      • A Berlis3,

        Affiliated with

        • W Janzarik4 and

          Affiliated with

          • B Volk1

            Affiliated with

            Diagnostic Pathology20072(Suppl 1):S24

            DOI: 10.1186/1746-1596-2-S1-S24

            Published: 14 March 2007

            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.

            Authors’ Affiliations

            (1)
            Abt. Neuropathologie, Universität Freiburg
            (2)
            Abt. Stereotaktische Neurochirurgie, Universität Freiburg
            (3)
            Abt. Neurochirurgie, Sektion Neuroradiologie, Universität Freiburg
            (4)
            Abt. Neurologie, Universität Freiburg

            Copyright

            © Mueller et al 2007

            This article is published under license to BioMed Central Ltd.

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