Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.
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.
Modan-Moses
D
D
Pediatric Endocrinology Service, Sheba Medical Center, Tel-Hashomer, Israel. molan@netvision.net.il
Weintraub
M
M
Meyerovitch
J
J
Segal-Lieberman
G
G
Bielora
B
B
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
D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone