Extended survival in stage IV adrenocortical carcinoma with recurrence and bone metastases

Adrenocortical carcinomas (ACCs) are rare aggressive endocrine tumours that commonly spread to the lymph nodes, liver and lung. Infrequently they metastasise to the bone and when present are often fatal.We report the case of a middle-aged male with a 23 cm ACC with extensive liver metastases at presentation. Index management was a two-stage procedure with resection of the primary lesion en bloc followed by hepatectomy with radiofrequency ablation to inoperable metastases. He had recurrence of disease with a left femur metastasis and additional liver metastases. Remission was obtained with radiotherapy, chemotherapy, bone resorptive inhibitors, mitotane and repeat radiofrequency ablation. He was discharged from regular surveillance and remains alive and free of active disease 14 years after initial diagnosis. To our knowledge, this is the longest recorded survival of a patient with stage IV ACC with recurrent disease and bone metastases.