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

Case report

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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.

Article Information

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.

© AANS, except where prohibited by US copyright law.

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    Brain MRI studies showing the right-sided pontine lesion that is isointense on T1-weighted imaging (A), hypointense on T2-weighted imaging (B), and shows intense enhancement on contrast administration (C and D).

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    Photomicrographs of tumor sections. A: Medium-power micrograph showing a polymorphic population of cells composed of pleomorphic tumor cells intermixed with lymphocytes, eosinophils, and occasional plasma cells. The larger pleomorphic cells range from round to oval to spindle shaped, and have large single to multiple vesicular nuclei with prominent nucleoli. H & E, original magnification ×450. B and C: High-power photomicrographs showing diffuse strong granular cytoplasmic positivity for CD1a (B) and CD68 (C) by the larger pleomorphic cells. Peroxidase antiperoxidase, original magnification ×450. D: High-power photomicrograph showing many of the larger pleomorphic cells, which have strong diffuse cytoplasmic positivity for S100. Peroxidase antiperoxidase, original magnification ×450. E: Medium-power photomicrograph showing a few scattered glial fibrillary acidic protein–positive cells among the other cells of the tumor indicating residual glial cells. Peroxidase antiperoxidase, original magnification ×300.

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    Brain MRI studies obtained with contrast enhancement showing axial image with lesion at presentation (A), follow-up axial image at 2 months showing almost doubling of the lesion (B), and axial (C) and sagittal (D) cuts showing response of the lesion to chemotherapy at 1-year follow-up.

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