MP61-10 CATASTROPHIC EVENTS RELATED TO TUBEROUS SCLEROSIS COMPLEX ARE UNLIKELY IN THOSE UNDERGOING ROUTINE SURVEILLANCE

Abstract

You have accessJournal of UrologyKidney Cancer: Epidemiology & Evaluation/Staging/Surveillance III (MP61)1 Sep 2021MP61-10 CATASTROPHIC EVENTS RELATED TO TUBEROUS SCLEROSIS COMPLEX ARE UNLIKELY IN THOSE UNDERGOING ROUTINE SURVEILLANCE Vanessa Lukas, Lauren Neal, Sarah Mccain, Mary Silvia, Jane Boggs, Theodore Stem, Matthew Miles, Roy Strowd, and Ram Pathak Vanessa LukasVanessa Lukas More articles by this author , Lauren NealLauren Neal More articles by this author , Sarah MccainSarah Mccain More articles by this author , Mary SilviaMary Silvia More articles by this author , Jane BoggsJane Boggs More articles by this author , Theodore StemTheodore Stem More articles by this author , Matthew MilesMatthew Miles More articles by this author , Roy StrowdRoy Strowd More articles by this author , and Ram PathakRam Pathak More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002101.10AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Tuberous Sclerosis Complex (TSC) is multi-system disorder characterized by epilepsy, cognitive dysfunction, and benign tumors of the eye, brain, heart, lung, and kidneys. TSC varies greatly in its severity and surveillance is necessary to monitor disease progression. We sought to characterize the prevalence of TSC-related catastrophic events as they relate to renal disease and determine the impact of distance of the patient’s residence to the site of operative services. METHODS: A retrospective analysis of patients afflicted with TSC was performed. Qualitative data for demographics, clinical manifestations of TSC, and renal specific characteristics were compiled. Only complete datasets were incorporated in the analysis. RESULTS: Complete data was available for 44 patients with mean age 35.6 years±13.8 (39% male, 82% urban). Distance traveled to clinic based on residential zip code was 30%, 41%, and 29% for >60 miles, 30-60 miles, and <30 miles, respectively. Frequency of TSC clinical manifestations included 86% with epilepsy, 84% with skin involvement, 80% with renal involvement, 80% with neurological involvement, 45% with pulmonary and/or cardiac involvement, 18% with lymphangioleiomyomatosis, and 14% with retinal hamartomas. Of those with renal manifestations, 77% had >10 angiomyolipomas (AML) at baseline imaging. The number of AMLs and estimated GFR did not change significantly over the median follow-up of 38 months. However, a median increase of 4mm (largest diameter) was observed for the dominant lesion (p-value=0.032). For those with renal manifestations, rural vs. urban status and distance from clinic did not influence adherence to surveillance (p-values >0.05). Catastrophic events related to renal manifestation of TSC were uncommon with 1 case of renal hemorrhage and 1 case of renal cell carcinoma. CONCLUSIONS: In this representative cohort of adult TSC patients, the most common manifestations include epilepsy, dermatologic, neurological, and renal manifestations. Increasing distance from clinic did not influence surveillance adherence. Catastrophic events occurred rarely and as such living near to emergency operative services is not likely to be necessary. Source of Funding: Internal institutional grant © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e1086-e1087 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Vanessa Lukas More articles by this author Lauren Neal More articles by this author Sarah Mccain More articles by this author Mary Silvia More articles by this author Jane Boggs More articles by this author Theodore Stem More articles by this author Matthew Miles More articles by this author Roy Strowd More articles by this author Ram Pathak More articles by this author Expand All Advertisement Loading ...