Emergent presentation of Langerhans cell histiocytosis in a pediatric patient: Acute cerebellar involvement causing obstructive hydrocephalus requiring posterior fossa decompression

Abstract

Langerhans cell histiocytosis (LCH) is a disorder of the monocyte-macrophage system that can be unifocal or systemic. Here, we present a pediatric case who initially presented with osseous LCH but again presented 6 years later emergently with cerebellar symptoms, cerebellar mass and obstructive hydrocephalus. Patient underwent biopsy of the cerebellum which was path proven intracranial LCH.

Keywords

Emergent decompression - intracranial Langerhans cell histiocytosis - obstructive hydrocephalus

• References

• 1 Grois NG, Favara BE, Mostbeck GH, Prayer D. Central nervous system disease in Langerhans Cell Histiocytosis. Hematol Oncol Clin North Am 1998;12:287-305.
• 2 Gabbay LB, Leite Cda C, Andriola RS, Pinho Pda C, Lucato LT. Histiocytosis: A review focusing on neuroimaging. Arg Neuropsiquiatr. 2014;72:548-58.
• 3 Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ. MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 2004;25:880-91.
• 4 Grois N, Prayer D, Prosch H, Lassmann H; CNS LCH Co-operative Group. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 2005;128:829-38.
• 5 Langerhans Cell Histiocytosis Treatment–Health Professional Version (PDQ ®). National Cancer Institute. http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq.
• 6 Wnorowski M, Prosch H, Prayer D, Janssen G, Gadner H, Grois N. Pattern and course of neurodegeneration in Langerhans Cell Histiocytosis. J Pediatr 2008;153:127-32.