Journal of Endocrinological Investigation

, Volume 24, Issue 8, pp 612–617 | Cite as

Hypopituitarism in Langerhans cell histiocytosis: Seven cases and literature review

  • Dalit Modan-Moses
  • M. Weintraub
  • J. Meyerovitch
  • G. Segal-Lieberman
  • B. Bielorai
  • I. Shimon
Case Report


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 hypothalamic-pituitary 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.


Langerhans cell histiocytosis hypopituitarism diabetes insipidus growth hormone deficiency hypocortisolism hypothyroidism obesity 


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Copyright information

© Italian Society of Endocrinology (SIE) 2001

Authors and Affiliations

  • Dalit Modan-Moses
    • 1
    • 2
  • M. Weintraub
    • 2
    • 3
  • J. Meyerovitch
    • 1
    • 2
  • G. Segal-Lieberman
    • 2
    • 4
  • B. Bielorai
    • 2
    • 3
  • I. Shimon
    • 2
    • 4
  1. 1.Pediatric Endocrinology ServiceSheba Medical CenterTel-HashomerIsrael
  2. 2.Sackler School of MedicineTel-Aviv UniversityTel-AvivIsrael
  3. 3.Department of Pediatric Hematology-OncologySheba Medical CenterTel-HashomerIsrael
  4. 4.Institute of EndocrinologySheba Medical CenterTel-HashomerIsrael

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