J Korean Soc Pediatr Endocrinol.  2004 Jun;9(1):72-75.

A Case of Langerhans Cell Histiocytosis Preceding with Central Diabetes Insipidus

• Kim JS
• Yoon WS
• Kim GM
• Choi YK
• Park KW
• Kim BH
• Yoo YS

Abstract

A 3-year-old girl presented with polydipsia and polyuria for last 2 years. Her fluid intake was 7~8 L/day, and urinalysis showed low osmolality and specific gravity. Central diabetes insipidus (DI) was diagnosed by a water deprivation test. Intranasal 1-desamino-8-D-arginine vasopressin relieved her symptoms and normalized urinary concentrations. A T1-weighted MRI scan revealed a symmetrical thickening of the central part of the pituitary stalk. Six months after the diagnosis of central DI, she developed papular skin lesions on her forehead. The lesions were surgically removed, and histologically classified as Langerhans cell histiocytosis (LCH). We concluded that thickening of the central part of the pituitary stalk might represent the first manifestation of LCH clinically presenting with central DI. In children with central DI, special attention should be paid to the appearance of the pituitary stalk using MRI for the various manifestations of LCH in the central nervous system.

Keyword

Pituitary stalk thickening; Central diabetes insipidus; Langerhans cell histiocytosis; Skin lesions; MRI

MeSH Terms

Central Nervous System
Child
Child, Preschool
Diabetes Insipidus, Neurogenic*
Diagnosis
Female
Forehead
Histiocytosis, Langerhans-Cell*
Humans
Magnetic Resonance Imaging
Osmolar Concentration
Pituitary Gland
Polydipsia
Polyuria
Skin
Specific Gravity
Urinalysis
Vasopressins
Water Deprivation
Vasopressins