Huge Langerhans cell histiocytosis granuloma of choroid plexus in a child with Hand-Schüller-Christian disease. Case report.
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.
D001706:Biopsy; D001921:Brain / Q000473:pathology / Q000601:surgery; D002648:Child; D002831:Choroid Plexus / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012086:Reoperation