Neuroimaging of Langerhans cell histiocytosis lesions

These examples demonstrate typical manifestations of LCH CNS and spinal cord lesions.
(A) Brain MRI demonstrates T2-hyperintensity in cerebellum classic for LCH neurodegenerative syndrome. In this case, the patient had radiologic and clinical response to treatment with cytarabine.
(B) Spinal MRI demonstrates significant spinal cord lesions. This is a somewhat atypical case of a 13-year-old girl who had marginal response to cytarabine, then clofarabine. BRAF-V600E was detected in cells from the CSF, and the patient ultimately had radiologic and clinical response to vemurafenib.
(C) Brain MRI demonstrates a pituitary mass classic for LCH, though differential diagnosis also includes germinoma, lymphoma, and pituitary hypophysitis. In this case, the lesion was biopsy proven to be LCH, and the patient responded to cytarabine therapy.