This research is being done because there is no treatment that will cure this type of cancer. Although some types of chemotherapy can cause this cancer to shrink for a time, better options are needed.
Name: Sunitinib
Description: 50 mg PO daily for 28 days, q 6 weeks (1 cycle=6 weeks or 42 days)Type: DrugSunitinib
Name: Temsirolimus
Description: 25 mg IV weekly (Days 1, 8, 15, 22, 29, 36), q 6 weeks (1 cycle=6 weeks or 42 days)Type: DrugTemsirolimus
Description: Objective response as assessed by RECIST version 1.1 criteria as a 30% decrease in the sum of the longest diameters of the target lesions (partial response) maintained for at least 4 weeks, or complete disappearance of disease and cancer related symptoms (complete response), also maintained for at least 4 weeks. Early progression is defined as progressive disease at or prior to the first assessment. The 95% confidence interval for response rate will be calculated. The median and range of the duration of response will be assessed
Measure: Objective Response Time: Every 4 weeksDescription: Response duration: median and range Time to progression: median, 95% confidence interval Progression free survival: median, 95% confidence interval Overall survival: median, 95% confidence interval Comparison of the time to first and second progression for patients who receive sunitinib and temsirolimus in sequence.
Measure: Efficacy Outcomes Time: 48 monthsDescription: Primary tumour tissue specimens and baseline blood samples, will be obtained from all subjects prior to first dose for genetic analysis and other evaluation. In addition, optional for responding patients will be a fresh tumour biopsy at time of progression and also optional for patients entered in the second stage of accrual for any disease cohort will be fresh tumour biopsy at baseline.
Measure: Translational Research Time: 48 monthsDescription: Adverse events will be monitored on an ongoing basis by the central office and their frequencies reported annually at investigators' meetings.
Measure: Safety Monitoring Time: Daily up to an expected average of 4 weeks after treatmentAllocation: Non-Randomized
Parallel Assignment
There is one SNP
Adverse events will be monitored on an ongoing basis by the central office and their frequencies reported annually at investigators' meetings.. Eligibility Criteria - ALL Patients Patients must have histologically or cytologically confirmed diagnosis of a rare tumour as follows: 1. Vascular sarcomas: Angiosarcoma, hemangiosarcoma, hemangiopericytoma, hemangioblastomas; 2. Clear cell carcinomas of the ovary, endometrium; 3. Medullary thyroid carcinoma; 4. Neuroendocrine tumours: paragangliomas and pheochromocytomas only; 5. Adrenocorticocarcinoma; 6. Thymic carcinoma; 7. Fibrolamellar hepatocellular carcinoma; 8. Exploratory genetic cohort for sunitinib: Rare tumours with somatic or germline mutations in sunitinib targets such as VEGFR, PDGFR, KIT, RET; 9. Exploratory genetic cohort for temsirolimus: Rare tumours arising from known or suspected germline mutations in mTOR pathway such as PTEN, TS1/2, LKB1, NF1/2 or somatic mutations in the mTOR pathway such as mutation or amplification of P13K or AKT; 10. --- P13K ---
rifampin phenytoin rifabutin St. John's wort carbamazepine efavirenz phenobarbital tipranavir Eligibility Criteria - ALL Patients Patients must have histologically or cytologically confirmed diagnosis of a rare tumour as follows: 1. Vascular sarcomas: Angiosarcoma, hemangiosarcoma, hemangiopericytoma, hemangioblastomas; 2. Clear cell carcinomas of the ovary, endometrium; 3. Medullary thyroid carcinoma; 4. Neuroendocrine tumours: paragangliomas and pheochromocytomas only; 5. Adrenocorticocarcinoma; 6. Thymic carcinoma; 7. Fibrolamellar hepatocellular carcinoma; 8. Exploratory genetic cohort for sunitinib: Rare tumours with somatic or germline mutations in sunitinib targets such as VEGFR, PDGFR, KIT, RET; 9. Exploratory genetic cohort for temsirolimus: Rare tumours arising from known or suspected germline mutations in mTOR pathway such as PTEN, TS1/2, LKB1, NF1/2 or somatic mutations in the mTOR pathway such as mutation or amplification of P13K or AKT; 10. --- P13K ---