MP42-18 MANAGEMENT OF HEREDITARY LEIOMYOMATOSIS AND RENAL CELL CANCER (HLRCC)-ASSOCIATED RENAL CELL CARCINOMA

Abstract

You have accessJournal of UrologyKidney Cancer: Localized: Surgical Therapy III (MP42)1 Sep 2021MP42-18 MANAGEMENT OF HEREDITARY LEIOMYOMATOSIS AND RENAL CELL CANCER (HLRCC)-ASSOCIATED RENAL CELL CARCINOMA Alexis Rompre-Brodeur, Jeunice Owens-Walton, Sandeep Gurram, Patrick T Gomella, Bradley R Webster, Beth Ryan, Deborah Nielson, Christopher J Ricketts, Jeffrey W Nix, Gennady Bratslavsky, Adam R Metwalli, Rabindra Gautam, Ashkan Malayeri, Maria J Merino, Peter A Pinto, Ramaprasad Srinivasan, Mark W Ball, and W Marston Linehan Alexis Rompre-BrodeurAlexis Rompre-Brodeur More articles by this author , Jeunice Owens-WaltonJeunice Owens-Walton More articles by this author , Sandeep GurramSandeep Gurram More articles by this author , Patrick T GomellaPatrick T Gomella More articles by this author , Bradley R WebsterBradley R Webster More articles by this author , Beth RyanBeth Ryan More articles by this author , Deborah NielsonDeborah Nielson More articles by this author , Christopher J RickettsChristopher J Ricketts More articles by this author , Jeffrey W NixJeffrey W Nix More articles by this author , Gennady BratslavskyGennady Bratslavsky More articles by this author , Adam R MetwalliAdam R Metwalli More articles by this author , Rabindra GautamRabindra Gautam More articles by this author , Ashkan MalayeriAshkan Malayeri More articles by this author , Maria J MerinoMaria J Merino More articles by this author , Peter A PintoPeter A Pinto More articles by this author , Ramaprasad SrinivasanRamaprasad Srinivasan More articles by this author , Mark W BallMark W Ball More articles by this author , and W Marston LinehanW Marston Linehan More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002063.18AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) is a hereditary condition in which affected individuals are at risk for the development of renal cysts, renal tumors and renal cysts with mixed cystic/solid tumors. The renal tumors can be an aggressive, early onset form of renal cell carcinoma (RCC) which has a propensity to metastasize when the primary tumors are small. We report our clinical experience in management of patients who presented with known or suspected HLRCC-associated RCC. METHODS: Patients with HLRCC-associated renal tumors who had surgical management at our institution between 1989 and 2021 were reviewed. Survival time to endpoint was estimated with a Kaplan-Meier method. RESULTS: Sixty-four patients underwent 90 renal surgeries for HLRCC-associated renal tumor. The median age at first renal surgery was 42.9 years (range 18-78 years) and median follow-up period was 58 months. All tested patients (61/64) had documented fumarate hydratase germline pathogenic variation. Two patients (3%) were in their teens, nine (14%) in their twenty’s and three (5%) had their first tumor in their seventies. Twenty-nine patients (45%) had multifocal disease, fifteen (23%) had bilateral disease. Four patients (6%) presented de novo with bilateral multifocal disease, at a median age of 34 years old. Nine patients (14%) subsequently developed additional new renal tumors without disease progression with a median renal tumor free interval of 213 months (95% CI 147-NR). The renal tumors, which were predominantly type 2 papillary renal cell carcinoma, have a propensity to metastasize even when as small as 0.5cm. As the tumors are often infiltrative into the normal renal parenchyma and through the renal capsule, we performed most with open surgical procedures with wide surgical margins. CONCLUSIONS: HLRCC-associated kidney cancers are clinically aggressive and can be early onset, bilateral and multifocal. We recommend lifelong annual MRI imaging starting at age 8 and we do not recommend active surveillance when even a small tumor is identified. When surgery is indicated, we recommend an open procedure with wide margins and intraoperative ultrasound to search for multifocal disease. Source of Funding: NA © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e779-e779 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Alexis Rompre-Brodeur More articles by this author Jeunice Owens-Walton More articles by this author Sandeep Gurram More articles by this author Patrick T Gomella More articles by this author Bradley R Webster More articles by this author Beth Ryan More articles by this author Deborah Nielson More articles by this author Christopher J Ricketts More articles by this author Jeffrey W Nix More articles by this author Gennady Bratslavsky More articles by this author Adam R Metwalli More articles by this author Rabindra Gautam More articles by this author Ashkan Malayeri More articles by this author Maria J Merino More articles by this author Peter A Pinto More articles by this author Ramaprasad Srinivasan More articles by this author Mark W Ball More articles by this author W Marston Linehan More articles by this author Expand All Advertisement Loading ...