Langerhans Cell Histiocytosis of Skull Base: A Rare Cause of Central Diabetes Insipidus in Young Adults

Abstract Langerhans cell histiocytosis (LCH) is a rare disorder that can affect multiple organs, including the brain. One of the most common manifestations of LCH in the brain is involvement of the pituitary stalk, which can lead to central diabetes insipidus (CDI). We retrospectively reviewed the medical records of a series of patients with LCH who presented with CDI. The patients' clinicopathology features, laboratory findings, imaging studies, and treatment outcomes were investigated. The three patients were all young adults (22–38 years old) and presented with polyuria and polydipsia. MRI scans showed thickening of the pituitary stalk in all three, while common etiologies were ruled out. Biopsies of the pituitary stalk confirmed the diagnosis of LCH. The patients were further treated with chemotherapy and steroids, and their symptoms improved. This case series highlights the importance of considering LCH in the differential diagnosis of CDI, especially in young adults. Early diagnosis and treatment are essential to improve outcomes for patients with LCH.