Evaluation of the Efficacy of Addition of Progesterone to Standard Chemotherapy in Adrenocortical Carcinoma (ACC) (PESETA)

Inclusion Criteria: Histologically confirmed diagnosis of ACC Locally advanced or metastatic disease not amenable to radical surgery resection ECOG performance status 0-2 Effective contraception Life expectancy > 3 months Age > 18 years Adequate bone marrow reserve (neutrophils >1,000/mm3 and/or platelets >80,000/mm3) and organ function (including renal, liver and cardiac function) Be able to comply with the protocol procedures and provide written informed consent. Exclusion Criteria: History of recent or active prior malignancy, except for cured non-melanoma skin cancer, cured in situ cervical carcinoma, breast ductal carcinoma in situ, or other treated malignancies where there has been no evidence of disease for at least 2 years Renal insufficiency (estimated glomerular filtration rate [GFR]<50 mL/min/1.73 m2) or significant liver insufficiency (serum bilirubin>2 times the upper normal range and/or serum alanine aminotransferase [ALT] or aspartate aminotransferase [AST]>3 times the upper normal range). GFRs will be calculated according to the validated formula (MDRD) Pregnancy or breast feeding Congestive heart failure (ejection fraction<45%) Preexisting grade 2 peripheral neuropathy Previous or current treatment with mitotane or other antineoplastic drugs for ACC Previous radiotherapy for ACC Any other severe acute or chronic medical or psychiatric condition or laboratory abnormality that would impart, in the judgment of the investigator, excess risk associated with study participation or study drug administration or that, in the judgment of the investigator, would make the patient inappropriate for entry into this study.