Functional ectopic adrenocortical carcinoma : a case report

BACKGROUND: Adrenocortical carcinomas (ACC) are rare malignant tumours, with a quoted incidence of 0.7–2.0 cases/million habitants/year, with ectopic ACC being exceptionally rate. Diagnosis is based on clinical and biological assessment supplemented by imaging. Patients commonly present with features secondary to steroid excess, but around 15% of patients are diagnosed incidentally.CASE REPORT: A 54-year old lady, presented to clinic for assessment of possible polycystic ovarian syndrome (PCOS). The patient gave a few months history of facial hirsutism accompanied by excessive weight gain and new onset hypertension. On further questioning, she had more sinister symptoms including easy bruising, proximal myopathy and insomnia. Initial investigations revealed a 0900 h cortisol of 867 nmol/l, testosterone 6.73 nmol/l, oestradiol of 129 pmol/l, DHEAS 2.50 umol/l, Androstenedione 9 (0.35-2.49) ng/ml, 17-hydroxyprogesterone of 24.8 ng/ml. 0900 h Cortisol post over-night Dexamethasone suppression test was 1482 nmol/l, ACTH was undetectable and 24-hour urinary cortisol >4138 nmol/24 hr (