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J Neurosurg Pediatr. 2013 Sep;12(3):258-61. doi: 10.3171/2013.6.PEDS13132. Epub 2013 Jul 12.

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

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Departments of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India.


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.

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