Endocrine Abstracts

Cushing’s disease (CD) is a rare disorder caused by an increased secretion of adrenocorticotropin hormone (ACTH) from the anterior pituitary gland, usually as a result of pituitary adenoma. Transsphenoidal tumour resection is the best first line therapy option for these patients but 10–30% of them fail to achieve remission and need further treatment modalities to eliminate hypercortisolemia. Long-term outcomes and possible impacts on quality of life in these patients are still not well examined. We report a case of a 73-year-old patient who presented in 1982, as 38-year-old women with generalized malaise, facial fullness, increased skin pigmentation, hirsutism, hypertension, hyperglycemia and depression. Laboratory evaluation showed elevated serum levels of ACTH and cortisol with loss of diurnal variation and no suppression after overnight low-dose dexamethasone test. Computerized tomography (CT) scan revealed hypodense zone in posterior pituitary and no lesions in adrenal glands. The patient underwent transsphenoidal surgery which confirmed 5 mm pituitary tumour in posterior lobe with anterior spreading into normal tissue that inhibited the complete resection. Histological finding was ACTH – secreting microadenoma. During the postoperative course, patient developed diabetes insipidus; depression and high blood pressure were still present. Serum cortisol level was 832 nmol/l, ACTH 26.3 pmol/l (reference range:1.2–10.2 pmol/l). The patient consented to bilateral total adrenalectomy in November 1984. which showed adrenal hyperplasia. After second surgery, the symptoms of CD were diminished and steroid replacement therapy was started as following treatment. During time, patient developed hypothyroidism, electrolyte concentrations are kept in balance. ACTH levels were up to 194 pmol/l (with marked hyperpigmentation localized predominantly in face area), cortisolemia up to 579 nmol/l according to which the Hydrocortisone dose adjustments were made. Recent pituitary magnetic resonance imaging (MRI) demonstrated no signs for tumour recurrence. After 33 years of therapy, the evaluated quality of life in our patient is not significantly impaired.