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

Case report

Restricted access

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:

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.



  • View in gallery

    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).

  • View in gallery

    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.

  • View in gallery

    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.



Bansal DMarwaha RKTrehan AGupta VVarma N: Langerhans' cell histiocytosis: experience from a single center. Indian Pediatr 45:6856882008


Barthez MAAraujo EDonadieu J: Langerhans cell histiocytosis and the central nervous system in childhood: evolution and prognostic factors. Results of a collaborative study. J Child Neurol 15:1501562000


Bergmann MYuan YBrück WPalm KVRohkamm R: Solitary Langerhans cell histiocytosis lesion of the parietooccipital lobe: a case report and review of the literature. Clin Neurol Neurosurg 99:50551997


Cagli SOktar NDemirtas E: Langerhans' cell histiocytosis of the temporal lobe and pons. Br J Neurosurg 18:1741802004


Demaerel PVan Gool S: Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology 50:85922008


Dhall GFinlay JLDunkel IJEttinger LJKellie SJAllen JC: Analysis of outcome for patients with mass lesions of the central nervous system due to Langerhans cell histiocytosis treated with 2-chlorodeoxyadenosine. Pediatr Blood Cancer 50:72792008


Grois NFahrner BArceci RJHenter JIMcClain KLassmann H: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr 156:8738812010


Kujat CHermes MHeide GBäumler SFeiden W: [Intracranial manifestations of Langerhans-cell histiocytosis. Nuclear magnetic resonance findings.]. Radiologe 34:6486551994. (Ger)


Prayer DGrois NProsch HGadner HBarkovich AJ: MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 25:8808912004


Rodríguez-Pereira CBorrás-Moreno JMPesudo-Martínez JVVera-Román JM: Cerebral solitary Langerhans cell histiocytosis: report of two cases and review of the literature. Br J Neurosurg 19:1921972005


Vourtsi APapadopoulos AMoulopoulos LAXenellis JVlahos L: Langerhans cell histiocytosis with involvement of the pons: case report. Neuroradiology 40:1611631998




All Time Past Year Past 30 Days
Abstract Views 28 28 5
Full Text Views 24 24 5
PDF Downloads 117 117 23
EPUB Downloads 0 0 0


Google Scholar