Thomas Sanford1, Patrick T Gomella2, Rashid Siddiqui2, Daniel Su2, Julie Y An3, Gennady Bratslavsky1, Mark W Ball4, W Marston Linehan2, Adam R Metwalli5. 1. State University of New York Upstate Medical Center, Syracuse, NY; Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD. 2. Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD. 3. Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD; Center for Interventional Oncology, National Cancer Institute, National Institutes of Health, Bethesda, MD. 4. Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD. Electronic address: Mark.ball@nih.gov. 5. Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD; Division of Urology, Howard University, Washington DC.
Abstract
INTRODUCTION: Patients with a confirmed germline mutation in the von Hippel-Lindau (VHL) tumor suppressor gene have been followed at the National Cancer Institute since the 1980s. In this study, we identify VHL patients with pheochromocytoma and long-term follow-up to determine the best candidates for active surveillance and surgical resection. METHODS: A prospectively collected database of patients with a confirmed germline VHL mutation was reviewed to identify patients with a history of pheochromocytoma and at least 10 years of follow up. The presence of symptoms was assessed at the time of resection. Imaging data obtained at each clinic visit was reviewed to evaluate mass size and annual growth rate. Catecholamine data were reviewed to evaluate for data above the upper limit of the reference range. Masses that underwent imaging at least 3 months apart were considered in our surveillance cohort. RESULTS: Median follow up was 16.7 years. There was a size-dependent increase in catecholamine production (P<0.05). For 36 masses on active surveillance, growth rate increased exponentially from 0.03 cm/y when masses were <1 cm to 0.32 cm/y when masses were greater than 2 cm. Approximately 1/3 of patients developed another pheochromocytoma after initial resection with a median time of 7.9 years. Partial adrenalectomy was associated with no metastatic events and a steroid-free rate of 97%. CONCLUSION: Active surveillance is a safe strategy for management of VHL associated pheochromocytoma in masses less than 2 cm.
INTRODUCTION: Patients with a confirmed germline mutation in the von Hippel-Lindau (VHL) tumor suppressor gene have been followed at the National Cancer Institute since the 1980s. In this study, we identify VHL patients with pheochromocytoma and long-term follow-up to determine the best candidates for active surveillance and surgical resection. METHODS: A prospectively collected database of patients with a confirmed germline VHL mutation was reviewed to identify patients with a history of pheochromocytoma and at least 10 years of follow up. The presence of symptoms was assessed at the time of resection. Imaging data obtained at each clinic visit was reviewed to evaluate mass size and annual growth rate. Catecholamine data were reviewed to evaluate for data above the upper limit of the reference range. Masses that underwent imaging at least 3 months apart were considered in our surveillance cohort. RESULTS: Median follow up was 16.7 years. There was a size-dependent increase in catecholamine production (P<0.05). For 36 masses on active surveillance, growth rate increased exponentially from 0.03 cm/y when masses were <1 cm to 0.32 cm/y when masses were greater than 2 cm. Approximately 1/3 of patients developed another pheochromocytoma after initial resection with a median time of 7.9 years. Partial adrenalectomy was associated with no metastatic events and a steroid-free rate of 97%. CONCLUSION: Active surveillance is a safe strategy for management of VHL associated pheochromocytoma in masses less than 2 cm.
Authors: Thomas H Sanford; Benjamin Barckley Storey; William Marston Linehan; Craig A Rogers; Peter A Pinto; Gennady Bratslavsky Journal: BJU Int Date: 2010-08-19 Impact factor: 5.588
Authors: Rachel D Aufforth; Pooja Ramakant; Samira M Sadowski; Amit Mehta; Katarzyna Trebska-McGowan; Naris Nilubol; Karel Pacak; Electron Kebebew Journal: J Clin Endocrinol Metab Date: 2015-10-09 Impact factor: 5.958
Authors: Patrick T Gomella; Thomas H Sanford; Peter A Pinto; Gennady Bratslavsky; Adam R Metwalli; W Marston Linehan; Mark W Ball Journal: Urology Date: 2020-02-26 Impact factor: 2.649