Adrenocortical Carcinoma (ACC) is a rare and aggressive tumour, with an incidence around one or two per million. Patients usually present early with manifestation of abnormal hormone secretion, however, there are patients who present late with a mass effect or distant metastases. New adjuvant treatment options, even after complete tumour removal, are desired because postoperative disease-free survival at five years is only around 30%.
We describe a case of a 16-year-old girl diagnosed with adrenocortical carcinoma, who presented with pulmonary metastases and suffered several recurrences after resection and adjuvant chemotherapy. This patient came to receive up to eight different lines of chemotherapy. Survival after being diagnosed with the disease was four years.
Future advances in the management of ACC will probably be connected with better understanding of the molecular pathogenesis.
Definitely, the establishment of detailed guidelines with the purpose of optimizing therapy with mitotane in combination with other antineoplastic drugs is still a task to be done
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