Background: A 42-year-old woman presented to the neuroendocrine unit of a hospital with recent-onset polydipsia, polyuria and oligomenorrhea. She had no visual symptoms, head injury or history of malignancy.
Investigations: Measurement of serum sodium and osmolality (as well as urine osmolality) after water deprivation, both before and after desmopressin administration. Measurement of basal serum concentrations of pituitary hormones, insulin-like growth factor 1 and thyroid hormone, cosyntropin stimulation testing of adrenal function, and growth-hormone-releasing hormone-arginine stimulation testing. MRI of the pituitary, CT of the chest and abdomen, skeletal surveys, analyses of cerebrospinal fluid, serology and histologic examination of an excised, painful submandibular salivary gland.
Diagnosis: Central diabetes insipidus and anterior hypopituitarism secondary to Langerhans cell histiocytosis.
Management: Replacement therapies, including desmopressin, levothyroxine, cyclic estrogen with medroxyprogesterone, and growth hormone. The stalk lesion remained stable after 7 years without specific therapy.
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