Perioperative outcomes of syndromic paraganglioma and pheochromocytoma resection in patients with von Hippel-Lindau disease, multiple endocrine neoplasia type 2, or neurofibromatosis type 1

• Published in: Surgery 2017-12, Vol.162 (6), p.1259-1269
• Main Authors: Butz, James J., Yan, Qi, McKenzie, Travis J., Weingarten, Toby N., Cavalcante, Alexandre N., Bancos, Irina, Young, William F., Schroeder, Darrell R., Martin, David P., Sprung, Juraj
• Format: Article
• Language: English
• Subjects: Adrenal Gland Neoplasms - surgery; Adrenalectomy - methods; Adult; Female; Humans; Intraoperative Complications - epidemiology; Intraoperative Complications - etiology; Laparoscopy; Male; Middle Aged; Multiple Endocrine Neoplasia Type 2a - surgery; Neurofibromatosis 1 - surgery; Paraganglioma - surgery; Pheochromocytoma - surgery; Postoperative Complications - epidemiology; Postoperative Complications - etiology; Preoperative Care - methods; Retrospective Studies; Treatment Outcome; von Hippel-Lindau Disease - surgery
• ISSN: 0039-6060; 1532-7361
• DOI: 10.1016/j.surg.2017.08.002

Pheochromocytoma and/or paraganglioma associated with neurofibromatosis type 1, multiple endocrine neoplasia type 2A, and von Hippel-Lindau disease have different catecholamine biochemical phenotypes. We examined perioperative outcomes of pheochromocytoma/paraganglioma resection in 3 syndromic forms. Retrospective review of patients undergoing resection of syndromic pheochromocytoma/paraganglioma from 2000 through 2016. Eighty-one patients underwent pheochromocytoma/paraganglioma resection (multiple endocrine neoplasia type 2A, n = 36; neurofibromatosis type 1, n = 26; von Hippel-Lindau disease, n = 19). Tumor size differed across groups; patients with neurofibromatosis type 1 and von Hippel-Lindau disease had the largest tumors (P = .017). Larger tumor volumes correlated with higher urine 24-hour total metanephrine (r = 0.94, P < .001; r = 0.67, P = .033; and r = 0.89, P < .001 for multiple endocrine neoplasia type 2A, von Hippel-Lindau disease, and neurofibromatosis type 1, respectively). High adrenergic secretion (24-hour urine metanepinephrine) was found in neurofibromatosis type 1 (median, 861 μg/24 h), similar to that found in multiple endocrine neoplasia type 2A (median, 809 μg/24 h). The highest noradrenergic secretion (24-hour urine normetanephrine) occurred with von Hippel-Lindau disease (median, 4,598 μg/24 h), followed by neurofibromatosis type 1 and multiple endocrine neoplasia type 2A (median, 1,607 and 923 μg/24 h, respectively). The highest graded complications occurred among patients with neurofibromatosis type 1 (P = .036). However, when comparing postoperative outcomes across 3 groups in those who had laparoscopic resection, there was no significant difference (P = .955). Patients with neurofibromatosis type 1 had the most volatile intraoperative hemodynamic course and more severe postoperative complications. These complications are related to large tumors associated with abundant catecholamine secretion and the fact that a high proportion underwent open resection. Among only patients who underwent laparoscopic procedures, there were no differences in postoperative outcomes across syndromic groups.