EVIDENCE SYNTHESIS The systematic review showed an absence of evidence for treating RKCs , with data only supporting sunitinib or MET inhibitors for some specific subtypes .
PATIENT SUMMARY Patients confronted with rare kidney cancers are often treated the same way as clear-cell renal cell carcinoma patients , despite little evidence from r and omized trials .
Molecular characterization of tumors to stratify patients may improve outcomes .
CONTEXT The European Association of Urology Renal Cell Carcinoma Guideline Panel recently conducted a systematic review of treatment options for patients with advanced non-clear-cell renal cell carcinomas ( RCCs ) , which showed a substantial lack of evidence for management recommendations .
OBJECTIVE To improve the outcomes of patients with rare kidney cancers ( RKCs ) , we performed a subsequent unstructured review to determine current treatment strategies and druggable pathways , involving key stakeholders with a global perspective to generate recommendations .
Phase II and biomarker study of the dual MET/VEGFR2 inhibitor foretinib in patients with papillary renal cell carcinoma.
PURPOSE Foretinib is an oral multikinase inhibitor targeting MET , VEGF , RON , AXL , and TIE-2 receptors . Activating mutations or amplifications in MET have been described in patients with papillary renal cell carcinoma ( PRCC ) . We aim ed to evaluate the efficacy and safety of foretinib in patients with PRCC . PATIENTS AND METHODS Patients were enrolled onto the study in two cohorts with different dosing schedules of foretinib : cohort A , 240 mg once per day on days 1 through 5 every 14 days ( intermittent arm ) ; cohort B , 80 mg daily ( daily dosing arm ) . Patients were stratified on the basis of MET pathway activation ( germline or somatic MET mutation , MET [ 7q31 ] amplification , or gain of chromosome 7 ) . The primary end point was overall response rate ( ORR ) . RESULTS Overall , 74 patients were enrolled , with 37 in each dosing cohort . ORR by Response Evaluation Criteria in Solid Tumors ( RECIST ) 1.0 was 13.5 % , median progression-free survival was 9.3 months , and median overall survival was not reached . The presence of a germline MET mutation was highly predictive of a response ( five of 10 v five of 57 patients with and without germline MET mutations , respectively ) . The most frequent adverse events of any grade associated with foretinib were fatigue , hypertension , gastrointestinal toxicities , and nonfatal pulmonary emboli . CONCLUSION Foretinib demonstrated activity in patients with advanced PRCC with a manageable toxicity profile and a high response rate in patients with germline MET mutations
EVIDENCE SYNTHESIS The systematic review showed an absence of evidence for treating RKCs , with data only supporting sunitinib or MET inhibitors for some specific subtypes .
PATIENT SUMMARY Patients confronted with rare kidney cancers are often treated the same way as clear-cell renal cell carcinoma patients , despite little evidence from r and omized trials .
Molecular characterization of tumors to stratify patients may improve outcomes .
CONTEXT The European Association of Urology Renal Cell Carcinoma Guideline Panel recently conducted a systematic review of treatment options for patients with advanced non-clear-cell renal cell carcinomas ( RCCs ) , which showed a substantial lack of evidence for management recommendations .
OBJECTIVE To improve the outcomes of patients with rare kidney cancers ( RKCs ) , we performed a subsequent unstructured review to determine current treatment strategies and druggable pathways , involving key stakeholders with a global perspective to generate recommendations .