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Kim, Lee, Kim, Lee, Koong, and Oh: A Case of Langerhans Cell Histiocytosis Presented with Central Diabetes Insipidus
Case Report
Journal of Korean Society of Endocrinology

Journal of Korean Society of Endocrinology 2005; 20(5): 513-518.

Published online: 20 October 2005

DOI: https://doi.org/10.3803/jkes.2005.20.5.513

A Case of Langerhans Cell Histiocytosis Presented with Central Diabetes Insipidus

Byung Chul Kim, Mi Yeoun Lee, Young Kue Kim1, Ok-Jun Lee2, Sung Soo Koong, Tae Keun Oh

Department of Internal Medicine, College of Medicine, Chungbuk National University, Cheong-ju, Korea.

1Department of Neurosurgey, College of Medicine, Chungbuk National University, Cheong-ju, Korea.

2Department of Pathology, College of Medicine, Chungbuk National University, Cheong-ju, Korea.

Received 25 August 2005       Accepted 4 October 2005

Copyright © 2005 Korean Endocrine Society

Abstract

Langerhans cell histiocytosis can cause central diabetes insipidus. Here, a case of Langerhans cell histiocytosis invading the pituitary stalk was experienced. The patient was 15 years old boy, with complaint of polydipsia and polyuria. A water deprivation test was carried out, and the urine osmolarity was increased from 165 to 469 mosm/kg following an injection of AVP to confirm the diagnosis of central diabetes insipidus. A pituitary function stimulation test gave a normal response. A sellar MRI was performed, which showed a thickened pituitary stalk mass (about 5.7 mm), with an increased size, 6.9 mm, on a second MRI 2 month later. A tissue biopsy was performed, which showed aggregates of histiocytes and inflammatory cells, with prominent eosinophils (H&E), and also revealed strong reactivity to anti-CD1a antibody on the immunohistochemistry. After confirmative tissue diagnosis, the patient received radiotherapy (900 cGy). The thickened mass of the pituitary stalk disappeared on the MRI following the radiotherapy. The patient was managed with DDAVP nasal spray, after which the polyuric symptoms were completely relieved.

Keywords: Langerhans cell histiocytosis, Central diabetes insipidus Water deprivation test

Figures and Tables

Fig. 1
(a) T1- weighted image of sellar MRI. This shows densely enhanced and thickened (diameter 5.7 mm) pituitary stalk. (b) The follow-up sellar MRI. This shows more thickened (diameter 6.9 mm) pituitary stalk than previous images.
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Fig. 2
(a) Histologic finding of pituitary mass (H&E staining ×200). This shows loose aggregates of histiocytes in a mixed inflammatory background with prominent eosinophils. (b) High power view reveals Langerhans cells with ovoid to reniform nuclei. (H&E, ×400)
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Fig. 3
Langerhans cells disclose strong immunoreactivity for anti-CD1a antibody. (×200, Immunohistochemistry)
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Fig. 4
The follow-up sellar MRI after radiotherapy. This shows that pituitary stalk mass disappeared.
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Table 1
Response to Water Deprivation test
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*arginine vasopressin 5U injection

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