Adrenocortical carcinoma evolving after diagnosis of preclinical Cushing'ssyndrome in an adrenal incidentaloma - Case report

A 43-year-old female patient underwent abdominal ultrasonography and CT sca n because of uncharacteristic abdominal pain. A 3-cm homogeneous adrenal tu mor was diagnosed, The endocrine tests revealed an adrenal preclinical Gush ing's syndrome (PCS), Due to the latent hormone excess we decided to operat e on the adrenal tumor. Since the tumor was small, laparoscopic adrenalecto my was performed. Histological evaluation showed an adrenocortical tumor of undetermined nature. Four months later the patient presented with a metast asizing cortisol- and androgen-producing adrenocortical carcinoma (ACC), Af ter pretreatment with ketoconazole to suppress the biosynthesis of adrenal steroids under substitution with hydrocortisone, we reduced the tumor load by surgery. Postoperatively we continued ketoconazole and started o,p'-dich lorodiphenyldichloroethane as well as chemotherapy with doxorubicin and sur amin, However, the patient died from ACC 7 months after adrenalectomy, It i s known from several reports that PCS may persist clinically silently or ma y progress to full-blown Gushing's syndrome. This is the first time a malig nant course of PCS is described, Independent of the initial therapeutic str ategy of PCS, i.e. surgery or regular follow-up visits, we must be aware th at also relatively small adrenal tumors can harbor malignancy.