Xanthogranuloma as an unsuspected cause of idiopathic central diabetes insipidus

To demonstrate that xanthogranuloma is a rare cause of idiopathic central diabetes insipidus in the early phase of the disease and that it presents as a suprasellar mass at a later stage. In addition, we emphasize the importance of identifying the cause of idiopathic central diabetes insipidus and review the literature concerning endocrine disturbance in central xanthogranuloma.

Review of recently published case reports of central xanthogranuloma with endocrine disorders. The case of a 35-year-old man who presented with a very large suprasellar mass is also reported. The patient was diagnosed with idiopathic central diabetes insipidus 20 years ago with normal brain magnetic resonance imaging.

Most cases of this disease present as supra- or parasellar masses with endocrine involvement, the most common of which (in approximately 75% of patients) is sex hormone deficiency. Diabetes insipidus was found in 65% of patients.

Xanthogranuloma should be in the differential diagnosis of idiopathic central diabetes insipidus and sellar and parasellar masses. A detailed skin examination is very important in making the diagnosis of central diabetes insipidus.