Endocrine Abstracts

Tumors of the adrenal cortex can present as incidental findings during abdominal imaging, as the cause of steroid excess and/or as malignancy. The most common adrenal disorder encountered today is the adrenal incidentaloma, which is mostly benign but can be associated with (subclinical) Cushing’s syndrome or primary aldosteronism. In contrast, adrenocortical carcinoma (ACC) represents a rare but highly malignant tumor entity. Over the last years studies including expressi...

Some elegant clinical observations of dysregulated cortisol-secretion in cases of ACTH-independent macronodular hyperplasia (AIMAH) and more rarely adrenocortical adenomas (ACA) have led to the concept of ‘aberrant’ or ‘illegitimate’ membrane receptors (AR) in adrenal Cushing syndrome. It this situation cortisol secretion is regulated by an extra-cellular ligand that usually does not stimulate cortisol secretion in normal adrenals. This abnormal response mi...

Adrenocortical carcinoma (ACC) is a rare tumour characterized by a dismal prognosis. The most important predictor of outcome is the possibility to attain a complete resection and prognosis is extremely poor when complete surgical removal of ACC is not feasible. Most patients have resectable disease at presentation; however, fully half of the patients who have undergone complete removal of the tumour are destined to relapse. The high recurrence rate of ACC has prompted many inv...

ACC is a rare, heterogeneous malignancy with poor prognosis. Data from the German ACC Registry (n=478) indicate a 5-year survival rate of 47%. In addition to mitotane, cytotoxic drugs are standard of care in advanced ACC. The best results have been reported by Berruti et al. for the combination of mitotane with etoposide, doxorubicin and cisplatin with an objective tumor response rate of 49% in 72 patients. A response rate of 36% was published for the combination...