The adrenocortical carcinoma (ACC) is a rare cancer of the adrenal cortex, affecting 0.5 to 2 cases per million inhabitants per year. The ACC is usually aggressive, with a 5-year survival of 25%. Most cases of ACC occur sporadically, but in some situations, this malignancy may be associated with a hereditary syndrome. It was reported the case of a 56 years old female patient, who initially presented with a mass in the upper pole of the left kidney, underwent to oncologic nephrectomy. Histopathological examination revealed adrenal cortex carcinoma. Initially, the treatment was mitotane, with the progression of the disease, it was initiated chemotherapy with doxorubicin, etoposide and cisplatin, associate d to mitotane. After five cycles, the disease was stable, however, there was toxicity and limit dose of anthracycline, the treatment was followed with cisplatin, etoposide and mitotane for another five cycles. Upon new restaging there was an increase in the number and size of nodules, it was started treatment with capecitabine, gemcitabine and mitotane. The patient received seven cycles, with good tolerance to treatment. The third line treatment showed clinical benefit for the patient, with prolonged survival and, improving the quality of life, and could be considered a routine practice for this disease. It is expected the emergence of new treatments grounded in the genetic and molecular profile, as well as predictor factors of response to the treatment, and waiting results of immunotherapy and other trials.
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