Clin Pediatr Hematol Oncol.  2006 Apr;13(1):72-79.

Central Diabetes Insipidus as a Manifestation of Langerhans Cell Histiocytosis: Report of 3 Cases with Literature Review

• Han DK
• Kook H
• Baek HJ
• Song ES
• Kang HS
• Kim MJ
• Kim SJ
• Kim SH
• Kim CJ
• Hwang TJ

Abstract

Central diabetes insipidus (CDI) is the most common manifestation of central nervous system involvement in Langerhans cell histiocytosis (LCH). Therefore, in patients with endocrinologically proven CDI, a comprehensive diagnostic evaluation is crucial to identify possible extracranial sites of LCH. However, once established, DI is mostly associated with life-long dependence on antidiuretic hormone (ADH) replacement therapy. We here report 3 cases of CDI in association with LCH, diagnosed by a water deprivation test. Two patients who presented with CDI as the initial manifestation showed thickened pituitary stalk on the sellar MRI. The 3rd patient developed CDI as a late sequela of LCH. Regression of pituitary thickness on MRI did not correlate with the clinical recovery of CDI, as shown in 2 cases.

Keyword

Langerhans cell histiocytosis; Diabetes insipidus; Pituitary stalk thickening; MRI

MeSH Terms

Central Nervous System
Diabetes Insipidus
Diabetes Insipidus, Neurogenic*
Histiocytosis, Langerhans-Cell*
Humans
Magnetic Resonance Imaging
Pituitary Gland
Water Deprivation