Functioning metastasis from adrenocortical carcinoma

Recurrence of Cushing's syndrome due to functional metastasis from adrenocortical carcinoma (ACC) after adrenalectomy is extremely rare. We describe a case of a 56-year-old woman who presented with Cushing's syndrome due to an ACC. Abdominal CT showed a heterogeneous left adrenal tumour of 8 cm as well as a right adrenal nodule with 3 cm. An 18F-fluorodeoxyglucose positron emission tomography-CT (18F-FDG/CT PET) revealed an increased uptake of both adrenal lesions (maximum standardised uptake values, 17.7 and 10.4 for left and right adrenal lesions, respectively). Patient underwent bilateral adrenalectomy with R0 resection. Pathological examination revealed a left ACC with a Weiss' score of 7, Ki67 10%, stage II European Network for the Study of Adrenal Tumours and a right adrenal adenoma. After surgery, clinical improvement was noted. Two months later, she noticed recurrence of hypercortisolism and multiple liver and lung metastasis were demonstrable, without evidence of local recurrence on the 18F-FDG/CT PET and abdominal MRI.