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ORLANDO, FL USA (UroToday.com) - Pheochromocytoma is more common in familial cancer syndromes, including von Hippel-Lindau (vHL) disease. The occurrence of pheochromocytoma in this population has been well described, however the natural history, management, and outcomes of vHL associated pheochromocytoma has not been well studied. The National Cancer Institute (NCI) has access to a unique population of patients and has attempted to characterize these findings based on their extensive experience.

auaThe authors from the NCI reviewed their vHL database from 1989 to 2012 and identified 88 vHL patients with a total of 135 lesions; 122 adrenal, 12 extra-adrenal and 1 metastatic pheochromocytoma. Clinical characteristics, surgical management and outcomes of these patients were collected and analyzed. Sixty-five (77%) of the patients were male, the average age of surgical intervention was 34 years and the average follow up was 10 years. The adrenal tumor growth rate was 0.29 cm/year and the extra-adrenal tumor growth rate was 0.27 cm/year. These patients were managed with active surveillance until such a point when the patient was symptomatic, the largest tumor was greater than or equal to 3 cm in size, or the patient had elevated catecholamines. Majority of the patients (54%) were managed with minimally invasive partial adrenalectomy, four patients required post-operative steroid replacement therapy and one patient presented with metastatic disease.

The authors conclude that pheochromocytomas in vHL patients tend to be slow growing and largely asymptomatic, therefore active surveillance in this patient population is both safe and effective with minimally invasive adrenal sparing surgery being the surgical intervention of choice.

Presented by Daniel Su at the American Urological Association (AUA) Annual Meeting - May 16 - 21, 2014 - Orlando, Florida USA

National Cancer Institute (NCI), Bethesda, MD USA

Written by Chinonyerem Okoro, University of California (Irvine), and medical writer for UroToday.com

AUA 2014, #AUA14, von Hippel-Lindau (vHL) disease, drenal pheochromocytoma


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