Isolated tumorous Langerhans cell histiocytosis of the brainstem: a diagnostic and therapeutic challenge

Case report

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  • 1 Departments of Neurosurgery,
  • 2 Pediatrics, and
  • 3 Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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Langerhans cell histiocytosis (LCH) of the CNS is a rare entity, known to involve primarily the hypothalamicpituitary region, with the clinical hallmark of diabetes insipidus. There have been a few reports of CNS LCH involving the brainstem as intraparenchymal enhancing lesions, but this has never been the presenting complaint of LCH. The authors report on a 7-year-old boy who presented with right cerebellopontine syndrome, in whom a well-defined, solid, enhancing lesion in the brainstem was diagnosed. Clinicoradiological differential diagnosis included glioma and tuberculosis. Biopsy revealed atypical histiocytes positive for CD68, CD1a, and S100 protein; these are the diagnostic features of LCH on histopathological examination. The rapid growth of the lesion was controlled with a chemotherapeutic regimen of cladribine.

Abbreviation used in this paper:LCH = Langerhans cell histiocytosis.

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Contributor Notes

Address correspondence to: Amey R. Savardekar, M.Ch., Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. email: ameysavardekar@gmail.com.

Please include this information when citing this paper: published online July 12, 2013; DOI: 10.3171/2013.6.PEDS13132.

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