Diagnosis and treatment of 8 patients with bilateral pheochromocytomas and paraganglioma

Zhu Qingguo; Zhu Wei; Ye Liefu; Hu Minxiong; He Yanyu; Gao Xiangxun

doi:10.3969/j.issn.2095-5146.2015.03.013

Abstract：Objective:To elucidate the diagnostic and therapeutic procedures of patients with bilateral pheochro-mocytomas and paraganglioma.Methods:The clinical data of 8 patients with bilateral pheochromocytomas and para-ganglioma from January 2008 to December 2012 were retrospectively analyzed.Hypertension was observed in 7 ca-ses.Another 1 case was admitted to hospital with abdomen masses through physical examination.Four cases were diagnosed as multiple endocrine neoplasia (MEN).Urinary VMA,and serum and urinary catecholamine were deter-mined in 8 cases.The level of urine VMA was elevated in 6 cases (75%)and serum or urinary catecholamine level elevated in 5 (62.5%).All patients were given colour Doppler ultrasound examination and CT scan.The positive rate of localization of colour Doppler ultrasound and CT was 87.5% and 100% respectively.All cases were given Doxazosin for two to four weeks preoperatively.Staged laparoscopic surgery was conducted on 4 cases.Simultaneous operations were performed with laparoscopic surgery via transperitoneal approach in 2 cases and via retroperitoneal approach in the rest 2 cases.Results:Paragangliomas were localized adjacent to the aorta (4 cases),adjacent to cava vein (2 cases),and at the renal hilus (2 cases).Laparoscopic procedures were successfully performed on all 8 cases. No one was converted to open surgery.Lymphatic leakage occurred after surgery in one case and lasted 2 weeks.The rest cases had no perioperative and postoperative complications.The diameter of the tumor was from 3 to 8 cm.The patients were discharged six to nine days postoperatively.During the follow-up period of 9 months to 4 years,only one case still suffered from hypertension.Acute adrenocortical insufficiency occurred after surgery in one case,and cured with a large dose of hormone.No tumor recurrence was found.Conclusions:Multiple endocrine neoplasia should be suspected when pheochromocytoma is bilateral and the patients family members also should be screened for hereditary syndromes.During operation for bilateral adrenal pheochromocytoma,multiple tumors and paraganglioma should be considered to avoid missing.Both the transperitoneal and retroperitoneal laparoscopic approaches are safe and feasible for bilateral pheochromocytoma with the advantage of minimal invasion.Long-term follow-up after oper-ation is necessary.