Cushings disease is relatively rare and affects most commonly adults aged 20 to 40. It is associated with high morbidity and mortality; early detection and diagnosis of Cushings disease may lead to decreased this two facts and improved quality of life.
We present a 32-year-old man with abdominal purple striae, central obesity with typical moon face and oscillatory blood pressure values.
Hormonal exams revealed normal plasmatic cortisol value but elevated urinary free cortisol (UFC) value - 350 μg/24 h(N=9-180). ACTH=10 pg/ml(N<46). Eye fundus and lateral skull X-ray were in normal range. Abdominal CT showed a 5 mm diameter left adrenal tumor. DXA-BMD spinal Z score=-2.2 S.D, Tscore =-2.2 S.D. The diagnosis was: Cushings syndrome (left adrenal adenoma), osteopenia, obesity. The first treatment was left adrenalectomy (histology: diffuse and nodular hyperplasia). After the first month post surgery: UFC=300 μg/24 h, abdominal echography: right adrenal hyperplasia. The new treatment was right adrenalectomy (histology: diffuse hyperplasia). After 3 weeks UFC =109.2 μg/24 h. After two months post surgery, the simptomatology persisted and the UFC level was high (511 μg/24 h); MRI - pituitary fosa with homogeneous content. Abdominal CT- glandular tissue (1 cm diameter) on the left adrenal locus. Surgical resection was performed for the remaining tissue, but the hypercortisolism persisted with ACTH=302 pg/ml. In order to exclude the ectopic ACTH syndrome, we performed also chest and abdominal CT, thyroid echography, chromogranin A and 5-Hydroxy-indolacetic acid. We decided that the pituitary surgery by transsphenoidal approach is the next step. ACTH post surgery =265 pg/ml; Cortisol pl.=639.2 mmoli/l. After 1 year, UFC =1545.8 nmoli/24 h, MRI pituitary tumor (8/6 mm). A new transsphenoidal resection was proposed (histology:acidophil pituitary adenoma). Plasmatic cortisol postsurgery =9 ng/ml. Long term treatment recomanded: Prednisone 5 mg/day, Fludrocortisone 0.1 mg/day.
The initial false normal level of ACTH and the variability of the normal ranges for plasmatic and urinary free cortisol depending on assay methodology created
the difficulties in diagnosing and managing Cushings disease.In our case, the clinical and biological parameters return to normal after 48 months since the first sign of the disease.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology