Erdheim-Chester Disease Mimicking an Intracranial Trigeminal Schwannoma: Case Report Publisher Pubmed



Alimohamadi M^{1, 2} ; Hartmann C³ ; Paterno V¹ ; Samii M^{1, 2} Show Affiliations
Source: Journal of Neurosurgery: Pediatrics Published:2015

Abstract

Erdheim-Chester disease (ECD) is non-Langerhans histiocytosis that can affect multiple organ systems. It usually affects middle-aged patients, and only a few reports of ECD in children appear in the literature. Central nervous system involvement is a common feature that usually occurs as infiltration of the hypothalamus-pituitary axis, cerebellum, and/ or brainstem. Meningeal involvement occurs less commonly. In this article, the authors discuss a rare pediatric case of ECD presenting as an infiltrative mass of the trigeminal nerve and resembling the clinical and imaging features of a trigeminal schwannoma. © 2015 AANS.