title: Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.
abstract: Central nervous system (CNS) involvement and, in particular, hypothalamic-pituitary involvement are well described features of Langerhans cell histiocytosis (LCH). The actual incidence of CNS-LCH disease is unknown and the natural history is poorly understood. Diabetes insipidus (DI) is reported to be the most common and well described manifestation of hypothalamic-pituitary involvement (up to 50%). Anterior pituitary dysfunction has been reported in up to 20% of patients with LCH, and occurs almost exclusively concurrently with DI. In the current paper we describe our experience with 7 patients (6 females and 1 male) in whom hypothalamicpituitary involvement was a major feature of LCH. Diagnosis was made in 4 patients during childhood or adolescence, and 3 patients were over 18 years old at the time of diagnosis. Our series exemplifies the wide spectrum of LCH-induced hypopituitarism, and demonstrates some unique features, including a higher incidence of CRH/ACTH deficiency compared to other reports (4/7 patients), and massive obesity in 2 of our patients. Endocrine function was not improved in any of our patients following medical treatment of LCH with chemotherapy and glucocorticoids. We conclude that pituitary-hypothalamic dysfunction is a common feature of LCH, and therefore all LCH patients should undergo a thorough endocrine evaluation periodically.
authors.affiliation: Pediatric Endocrinology Service, Sheba Medical Center, Tel-Hashomer, Israel. molan@netvision.net.il
authors.forename: D
authors.initials: D
authors.lastname: Modan-Moses
authors.forename: M
authors.initials: M
authors.lastname: Weintraub
authors.forename: J
authors.initials: J
authors.lastname: Meyerovitch
authors.forename: G
authors.initials: G
authors.lastname: Segal-Lieberman
authors.forename: B
authors.initials: B
authors.lastname: Bielora
mesh_terms: D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000172:deficiency; D000328:Adult; D002648:Child; D003346:Corticotropin-Releasing Hormone / Q000172:deficiency; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007018:Hypopituitarism / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009765:Obesity / Q000150:complications; D017211:Treatment Failure
chemical_list: D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone