Authors: Dalit ModanMoses M Weintraub J Meyerovitch G SegalLieberman B Bielorai I Shimon
Publish Date: 2014/03/11
Volume: 24, Issue: 8, Pages: 612-617
Abstract
Central nervous system CNS involvement and in particular hypothalamicpituitary involvement are well described features of Langerhans cell histiocytosis LCH The actual incidence of CNSLCH 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 hypothalamicpituitary 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 LCHinduced 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 pituitaryhypothalamic dysfunction is a common feature of LCH and therefore all LCH patients should undergo a thorough endocrine evaluation periodically
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