PD51-01 MANAGEMENT OF GENETICALLY DEFINED RENAL TUMORS UTILIZING SIZE-BASED RISK STRATIFICATION: LONG-TERM RESULTS

Abstract

You have accessJournal of UrologyKidney Cancer: Localized: Active Surveillance1 Apr 2018PD51-01 MANAGEMENT OF GENETICALLY DEFINED RENAL TUMORS UTILIZING SIZE-BASED RISK STRATIFICATION: LONG-TERM RESULTS Mark Ball, Julie An, James Peteron, Maria Merino, Ramaprasad Srinivasan, Adam Metwalli, and W. Marston Linehan Mark BallMark Ball More articles by this author , Julie AnJulie An More articles by this author , James PeteronJames Peteron More articles by this author , Maria MerinoMaria Merino More articles by this author , Ramaprasad SrinivasanRamaprasad Srinivasan More articles by this author , Adam MetwalliAdam Metwalli More articles by this author , and W. Marston LinehanW. Marston Linehan More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2018.02.2341AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Genetically defined renal cell carcinomas, including those associated with von Hippel-Lindau (VHL; clear cell RCC, VHL gene), Hereditary Papillary Renal Carcinoma (HPRC, Type 1 Papillary RCC, MET gene) Birt-Hogg-Dubé (BHD, chromophobe RCC, FLCN gene), Hereditary Leiomyomatosis and Renal Cell Carcinomas (HLRCC; Type 2 Papillary RCC, Fumarate Hydratase gene) and Succinate Dehydrogenase RCC (SDHRCC, eosinophilic RCC, SDHB gene) are managed at our institution based on genotype. While metastasis may develop with small (< 3 cm) HLRCC or SDHRCC renal tumors, based on early observations of low metastatic potential of small lesions renal lesions in VHL, HPRC and BHD, these patients have been managed with active surveillance until the largest tumor reaches 3 cm, at which time surgical resection of ipsilateral tumors is recommended. The present study was performed in order to evaluate the oncologic efficacy of the 3 cm size threshold with in a cohort of VHL, HPRC and BHD patients with long-term follow-up. METHODS Patients managed on prospective National Cancer Institute Institutional Review-approved protocols with VHL-, HPRC- and BHD-associated solid renal masses were identified. The diameter of the largest solid tumor, the length of follow-up, the presence of metastatic disease was evaluated. The proportion of patients who developed metastatic disease at size thresholds beyond 3 cm was assessed in 1 cm increments. Metastasis-free survival (MFS) was defined as the interval from initial screening to development of metastatic disease. RESULTS A total of 634 patients with genetically defined RCC were identified, including 440 with VHL-, 43 with HPRC- and 151 with BHD-associated renal tumors. While metastatic disease was found to have developed in 42 patients (10.1%) with VHL-, 6 (14%) with HPRC-, and 8 (5.3%) with BHD-associated RCC, to date, no patient who underwent surgical resection when the largest tumor was < 3 cm has yet developed metastatic disease. Table 1 lists the proportion of patients who developed metastases by size of their largest tumor. MFS for VHL patients whose renal tumors were resected when they reached 3 cm threshold was significantly longer compared to those who were not (p=0.007). The 5, 10 and 20-year MFS for patients whose tumors had not been resected when they reached the 3 cm threshold was 95.7%, 91.1%, and 69.5%, respectively. CONCLUSIONS In a cohort of 634 patients with VHL-, HPRC- and BHD-associated RCC adherence to the 3 cm guideline was associated with superior MFS compared to those who were not. In addition, these findings may have broad applicability for the management of non-hereditary genetically defined renal tumors. © 2018FiguresReferencesRelatedDetails Volume 199Issue 4SApril 2018Page: e975 Advertisement Copyright & Permissions© 2018MetricsAuthor Information Mark Ball More articles by this author Julie An More articles by this author James Peteron More articles by this author Maria Merino More articles by this author Ramaprasad Srinivasan More articles by this author Adam Metwalli More articles by this author W. Marston Linehan More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...