Huge Langerhans cell histiocytosis granuloma of choroid plexus in a child with Hand-Schüller-Christian disease

Abstract

In the literature, only two previous cases of presumed Langerhans cell histiocytosis (LCH) granuloma involving choroid plexus have been reported in patients with Hand-Schüller-Christian (HSC) disease, but those cases were not verified by immunohistochemistry or electron microscopy. The authors report a case of LCH granuloma of the choroid plexuses of bilateral lateral ventricles and the fourth ventricle in a child affected by HSC disease. This disease was confirmed by the presence of Birbeck granules on electron microscopy and positive reactivity to S-100 protein. Clinical, radiographic, and histological features are discussed. This case illustrates that LCH granuloma should be considered in the differential diagnosis of intraventricular masses.