TY - JOUR
T1 - Perioperative outcomes of syndromic paraganglioma and pheochromocytoma resection in patients with von Hippel-Lindau disease, multiple endocrine neoplasia type 2, or neurofibromatosis type 1
AU - Butz, James J.
AU - Yan, Qi
AU - McKenzie, Travis J.
AU - Weingarten, Toby N.
AU - Cavalcante, Alexandre N.
AU - Bancos, Irina
AU - Young, William F.
AU - Schroeder, Darrell R.
AU - Martin, David P.
AU - Sprung, Juraj
N1 - Funding Information:
Financial support was provided by the Department of Anesthesiology, Mayo Clinic, Rochester, MN. The authors have no conflicts of interest, financial or otherwise, to disclose. Original Communication
Publisher Copyright:
© 2017 Elsevier Inc.
PY - 2017/12
Y1 - 2017/12
N2 - Background 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. Methods Retrospective review of patients undergoing resection of syndromic pheochromocytoma/paraganglioma from 2000 through 2016. Results 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). Conclusion 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.
AB - Background 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. Methods Retrospective review of patients undergoing resection of syndromic pheochromocytoma/paraganglioma from 2000 through 2016. Results 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). Conclusion 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.
UR - http://www.scopus.com/inward/record.url?scp=85029451145&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85029451145&partnerID=8YFLogxK
U2 - 10.1016/j.surg.2017.08.002
DO - 10.1016/j.surg.2017.08.002
M3 - Article
C2 - 28919049
AN - SCOPUS:85029451145
VL - 162
SP - 1259
EP - 1269
JO - Surgery (United States)
JF - Surgery (United States)
SN - 0039-6060
IS - 6
ER -