PD1-05 ANALYSIS OF FACTORS ASSOCIATED WITH IMPROVEMENT OF METABOLIC DISORDERS FOLLOWING LAPAROSCOPIC ADRENALECTOMY IN PATIENTS WITH CUSHING′S SYNDROME

Abstract

INTRODUCTION AND OBJECTIVES: Association of pheochromocytoma with von Hippel-Lindau (vHL) disease is well described, these patients often present with no characteristic signs or symptoms of catecholamine abundance. Natural history, management, and outcome of this cohort of patients are not well studied. Data from vHL patients evaluated at the National Cancer Institute with pheochromocytoma and long duration follow up are analyzed and our management algorithm described. METHODS: From 2005 to 2013, data from our vHL database were retrospectively reviewed; patients with a long duration of follow up and who underwent surgical intervention involving pheochromocytoma were included. All patients were found to have positive mutations in the vHL gene and have pathologic confirmation of either adrenal or ectopic pheochromocytoma. Demographic data, pathology, tumor measurement, laboratory measurements including serum catecholamine levels, surgical management, and outcome data were collected and analyzed. RESULTS: 39 patients from 29 vHL families were identified, this cohort presented with pheochromocytoma at an average age of 34 years, 31 (79%) of the patients were male. Patients were followed for an average of 86 months (range, 21, 172) and had an average size of 2.78 cm (range, 1.1 e 4.5 cm) pheochromocytoma at intervention. One patient presented with a >20 cm pheochromocytoma and metastatic disease, no other patient in this cohort developed metastatic disease. Plasma fractionated normetanephrines were found to be the most commonly positive catecholamine level, in 31 (79.4%) of the patients at intervention. 92% of the patients underwent adrenal sparing surgery and 87% of patients underwent minimal invasive surgery. The most common trigger for surgery was elevated catecholamine levels (44%) and the second most common trigger was symptomatic pheochromocytoma (30%). The average growth rate for the adrenal pheochromocytoma was 0.23 cm/year, and all patients had a positive MIBG scan. CONCLUSIONS: The role of active surveillance in management of pheochromocytoma in the vHL population is not well studied. Adrenal pheochromocytoma in this population tends to be slow growing and asymptomatic. Majority of this cohort of patients can be safely monitor with serial imaging and catecholamine levels until such a point where either catecholamine levels are elevated or the patient becomes symptomatic. Minimally invasive adrenal sparing surgery is the intervention of choice.