Endocrine Abstracts

A 59-year-old lady presented with Cushing’s syndrome in 1991. Hormone testing revealed that it was due to ectopic secretion of ACTH. However, even after a comprehensive diagnostic workup, the source of ACTH secretion was not found. Since the symptoms (arterial hypertension and diabetes) were not successfully controlled with drugs, bilateral adrenalectomy was performed and the patient was put on hydrocortisone and fludrocortisone. The patient was then followed annually in the outpatient clinic. After 15 years of follow up, the ACTH levels started to rise: at first gradually and then duplicated in the year 2011. Morphologic diagnostic procedures, which became more accurate and easily accessible in the meantime, were repeated then. CT of the thorax revealed a 4 cm big tumorous formation in the left lower lung lobe. Unfortunately, we were not able to get a representative biopsy sample with bronchoscopy. The lesion was intermediate metabolically active on PET–CT and was positive for somatostatin receptors on octreoscan. Both studies were negative for possible secondary lesions in other parts of the body. The tumour was successfully removed with video assisted thoracic surgery and histologically confirmed to be a carcinoid. The cells of the tumor stained positive for ACTH. The control serum levels of ACTH were suppressed and stayed suppressed until now. In conclusion, we report a case of ectopic ACTH secreting tumour that was found after 20 years of follow-up and then successfully removed. Two things are important for planning care in the future and focusing possible research in such patients: i) since many tumours are small at least in the early stages, more accurate diagnostic methods provide a greater chance to find them. They should be repeated periodically (at least every few years). ii) It is important to efficiently control the cortisol excess, until the tumour is found.