RESUMO
BACKGROUND: Adrenocortical carcinoma (ACC), the second most aggressive malignant tumor, lacks epidemiological data worldwide; therefore, every new case can improve the understanding of the pathology and treatment of this malignancy. CASE SUMMARY: We present the case of a 66-year-old Caucasian woman with a giant androgen-producing ACC (21 cm × 17 cm × 12 cm; 2100 g), without metastases, which unusually presented with an acute onset of atrial flutter and congestive heart failure. The cardiac complications observed in our case support the hypothesis that androgen excess in women is a cardiovascular risk factor. Androgen excess in women can be a rare cause of reversible dilated cardiomyopathy, therefore a comprehensive approach to the patient is essential to improve the recognition of androgen-secreting ACC. The atrial flutter was remitted after initiation of drug treatment during admission. The severe heart failure was totally remitted at 6 mo after radical open surgery to remove the giant ACC. CONCLUSION: Radical open surgery to remove a giant androgen-producing ACC was the first-line treatment to cure the excess of androgen, which determined the total remission of cardiac complications at 6 mo after surgery in the women of this case report.