J Korean Soc Endocrinol.  2005 Oct;20(5):513-518. 10.3803/jkes.2005.20.5.513.

A Case of Langerhans Cell Histiocytosis Presented with Central Diabetes Insipidus

• Kim BC
• Lee MY
• Kim YK
• Lee OJ
• Koong SS
• Oh TK

• KMID: 2200600
• DOI: http://doi.org/10.3803/jkes.2005.20.5.513

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 5.7mm), 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.

MeSH Terms

Adolescent
Biopsy
Deamino Arginine Vasopressin
Diabetes Insipidus, Neurogenic*
Diagnosis
Eosinophils
Histiocytes
Histiocytosis, Langerhans-Cell*
Humans
Immunohistochemistry
Magnetic Resonance Imaging
Male
Osmolar Concentration
Pituitary Gland
Polydipsia
Polyuria
Radiotherapy
Water Deprivation
Deamino Arginine Vasopressin

Figure

• 

  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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