A retrospective cohort study of surgery for adrenocortical carcinoma in a tertiary endocrine surgery unit

INTRODUCTION: Adrenocortical cancer (ACC) is a rare endocrine malignancy with a variable prognosis. This study examines clinical outcomes of patients with ACC who underwent surgery in an endocrine surgery unit over 14 years. METHODS: Demographic, clinical, pathological and follow-up data on consecutive patients who underwent surgery for ACC or cortical tumours of uncertain malignant potential (UMP) and patients who had a clinical diagnosis of ACC were collected and descriptive analyses were conducted. RESULTS: Of 48 patients, 32 had ACC confirmed on histology, 10 had a clinical diagnosis and 6 had UMPs. Of those with a histological diagnosis, there were 12 men and 20 women with a median (range) age at first operation of 53 (16-77) years. The histologic type was oncocytic in eight (25%) patients and metastatic disease was present in six (19%) patients at time of operation. ENSAT staging was stage I in 2 (6%), stage II in 18 (56%), stage III in 5 (16%), stage IV in 6 (19%) and unknown in 1 (3%) patient. Complete resection was achieved in 16 (50%) patients. Adjuvant treatments included mitotane and external beam radiotherapy in 18 (56%) and 2 (6%) patients, respectively. Four patients (with initial surgery outside this centre) had multiple operations. The median (range) overall survival and median (range) time to persistent or recurrent disease was 33 (7-172) months and 8 (0-71) months in histology-proven ACC. CONCLUSIONS: This study provides a contemporary perspective on epidemiology and practice in the management of ACC in the UK.