Introduction: Adrenocortical carcinoma is a rare and aggressive tumor. The incidence is 0.7-2.0 new cases/million people/year. The treatment of choice and potentially curative is surgical excision, 50% of patients are admitted to radical surgery, but 40-70% of patients have recurrence after. 5-year survival for patients with distant metastases is 0% to 28%. Currently, only two patients are reported with long-term survival of 30 or more years with recurrent and metastatic adrenocortical carcinoma.

Case report: We describe, a female, 55 years old, who began 33 years ago with clinical and biochemical Cushing's syndrome, CT scan revealed a 7 cm nodule in the left adrenal gland. Left adrenalectomy was maintained with fragmentation of the tumor at the site followed by radiotherapy due to suspected invasion of the upper renal pole. After, there was no apparent evidence of local invasion or metastases and with a resolution of clinical symptoms and laboratory changes. After six years of disease-free survival, there is a recurrence of the clinical and biochemical Cushing's syndrome. CT scan showed liver damage in segment VII. Resection of hepatic segments VI to VIII was performed, confirming a secondary deposit of adrenocortical carcinoma. Ten years after the initial diagnosis, a new clinical recurrence occurred, a 7 cm peritoneal lesion adjacent to the ascending colon was detected by imaging, excised, and compatible with probable seeding in the site of the previous liver resection. Thirty years after the first intervention, during the third clinical and biochemical recurrence, a CT scan showed a new left intraperitoneal lesion, which was completely resected. Corresponding to a new metastasis of adrenocortical carcinoma. Thirty-three years after the diagnosis, she is normal weight, normotensive, and without radiological or clinical evidence of tumor.

Discussion: Establishing a prognosis in adrenocortical carcinoma remains a challenge, although many patients die months after diagnosis, exceptions exist as the case reported.