You have accessJournal of UrologyKidney Cancer: Epidemiology & Evaluation/Staging I1 Apr 2016MP73-15 METASTATIC PAPILLARY RENAL CANCER: DOES HEREDITARY ORIGIN IMPACT SURVIVAL? Abhinav Sidana, Meet Kadakia, Julia Friend, Akhil Muthigi, Louis Krane, Geri Hawks, Martha Ninos, W Martson Linehan, and Ramaprasad Srinivasan Abhinav SidanaAbhinav Sidana More articles by this author , Meet KadakiaMeet Kadakia More articles by this author , Julia FriendJulia Friend More articles by this author , Akhil MuthigiAkhil Muthigi More articles by this author , Louis KraneLouis Krane More articles by this author , Geri HawksGeri Hawks More articles by this author , Martha NinosMartha Ninos More articles by this author , W Martson LinehanW Martson Linehan More articles by this author , and Ramaprasad SrinivasanRamaprasad Srinivasan More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2016.02.1670AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Hereditary forms of renal cancer tend to have a younger age of onset but there is little data to address the impact of early onset on survival compared to patients with sporadic forms of metastatic papillary renal cell cancer (pRCC). The primary objective of our study is to compare overall survival between hereditary and sporadic forms of metastatic pRCC. The secondary objective is to identify predictors for overall survival in hereditary forms of metastatic pRCC. METHODS Retrospective evaluation of the medical records of patients with metastatic pRCC seen at the Urologic Oncology Branch, National Cancer Institute (2000-2014) was undertaken. Patient demographics, tumor characteristics and outcomes were studied. Overall survival of the groups was estimated by Kaplan-Meier survival analysis. Univariate Cox proportional hazard regression analysis was done to identify predictors of overall survival. RESULTS One hundred and six patients with metastatic pRCC were identified, of whom 53 (50.0%) had a hereditary variant (49 with Hereditary Leiomyomatosis and Renal Cell Cancer, 3 with Hereditary Papillary Renal Cell Carcinoma, and 1 with Birt-Hogg-Dube). In our cohort, metastatic hereditary pRCC were more likely to be papillary type 2 than sporadic forms (p<0.001). Hereditary pRCC patients presented (mean age 40.1 vs 54.7 years, p<0.001) and developed metastasis (mean age 42.0 vs 57.3 years, p<0.001) at a younger age than sporadic pRCC patients. No difference in tumor staging at presentation was found between the two groups. While mean time from primary diagnosis to metastasis was shorter in hereditary group (32.7 vs 45.7 months, p=0.446), the difference was not statistically significant. There was no difference in estimated median overall survival after development of metastasis (40.8 vs 35.1 months, p=0.801) between the two groups. Corrected calcium greater than 10 (p=0.012), elevated lactate dehydrogenase (p=0.010), neutrophil-lymphocyte ratio >5 (p=0.013) and platelet-lymphocyte ratio >210 (p=0.001) at the time of initiation of systemic therapy predicted worse overall survival in hereditary metastatic pRCC. CONCLUSIONS Hereditary pRCC patients presented and developed metastasis at a younger age than those with sporadic forms but the overall survival after the development of metastasis was comparable between two groups. Several traditional prognostic factors of survival identified in sporadic RCC also appear to predict survival in hereditary forms. To our knowledge, this is the first report detailing survival and prognostic factors in patients with hereditary metastatic pRCC. © 2016FiguresReferencesRelatedDetails Volume 195Issue 4SApril 2016Page: e966 Advertisement Copyright & Permissions© 2016MetricsAuthor Information Abhinav Sidana More articles by this author Meet Kadakia More articles by this author Julia Friend More articles by this author Akhil Muthigi More articles by this author Louis Krane More articles by this author Geri Hawks More articles by this author Martha Ninos More articles by this author W Martson Linehan More articles by this author Ramaprasad Srinivasan More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...