Orteronel (TAK-700) in Metastatic or Advanced Non-resectable Granulosa Cell Ovarian Tumors. The Greko II Study.

Inclusion Criteria: * Voluntary written informed consent. * Patients, even if surgically sterilized who: 1. Agree to practice effective barrier contraception during the entire study treatment period and for 4 months after the last dose of study drug, or 2. Agree to completely abstain from intercourse. * Patients 18 years or older. * Screening clinical laboratory values as specified below: * Serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) must be \<=2.5 X ULN. * Total bilirubin \<=1.5 X ULN. * Estimated creatinine clearance using the Cockcroft-Gault formula must be \>40 mL/minute. * Absolute neutrophil count (ANC) \>=1500/mcL and platelet count \>=100,000/mcL. * Histologically confirmed granulosa cell ovarian tumor with locally advanced non-resectable or metastatic disease, measurable or evaluable by RECIST. * Availability of sufficient biopsy material to confirm the malignant diagnosis of granulosa cell ovarian tumor by a centralized pathologist and to perform the determine the FOXL2 402C mutation → G (C134W). However study entry will be allowed based just on the histological local diagnosis. * Life expectancy \>=12 weeks * Screening calculated ejection fraction of greater than or equal to normal by multiple gated acquisition (MUGA) scan, or by echocardiogram (ECHO). * Stable medical condition, including the absence of acute exacerbations of chronic illnesses, serious infections, or major surgery within 4 weeks before first dose of study drug/randomization. Exclusion Criteria: * History of myocardial infarction, unstable symptomatic ischemic heart disease, ongoing arrhythmias of Grade \> 2 (NCI CTCAE, version 4.02)(56), thromboembolic events (eg, deep vein thrombosis, pulmonary embolism, or symptomatic cerebrovascular events), or any other cardiac condition (eg, pericardial effusion restrictive cardiomyopathy) within 6 months prior to first dose of study drug. Chronic stable atrial fibrillation on stable anticoagulant therapy is allowed. * New York Heart Association Class III or IV heart failure. * ECG abnormalities of: 1. Q-wave infarction, unless identified 6 or more months prior to screening 2. QTc interval \> 460 msec * Female subject is pregnant or breast-feeding. Confirmation that the subject is not pregnant must be established by a negative serum ?- human chorionic gonadotropin (?-hCG) pregnancy test result obtained during screening. * Patient has received other investigational drugs within 28 days before enrollment * Diagnosed or treated for another malignancy within 2 years of enrollment, with the exception of complete resection of basal cell carcinoma or squamous cell carcinoma of the skin, an in situ malignancy. * Prior therapy with orteronel, ketoconazole, abiraterone, aminoglutethimide or enzalutamide. * Patients received radical radiotherapy \<= 4 weeks before starting the study treatment or who have not recovered from the toxicities of radiotherapy. Palliative radiotherapy of painful bone lesions is allowed up to 14 days before the start of study treatment. * Known hypersensitivity to compounds related to orteronel or to orteronel excipients. * Uncontrolled hypertension despite appropriate medical therapy (BP of greater than 160 mmHg systolic and 90 mmHg diastolic at 2 separate measurements no more than 60 minutes apart during the Screening visit). Note: patients may be rescreened after adjustment of antihypertensive medications. * Known active chronic hepatitis B or C, life-threatening illness unrelated to cancer, or any serious medical or psychiatric illness that could, in the investigator?s opinion, potentially interfere with participation in this study. * Likely inability to comply with the protocol or cooperate fully with the investigator and site personnel. * Known gastrointestinal (GI) disease or GI procedure that could interfere with the GI absorption or tolerance of orteronel, including difficulty swallowing tablets.