双侧肾上腺嗜铬细胞瘤并副神经节瘤8例临床诊治

Diagnosis and treatment of 8 patients with bilateral pheochromocytomas and paraganglioma

目的:探讨双侧肾上腺嗜铬细胞瘤(PHEO)并副神经节瘤(PGL)的诊治经验。方法:采用回顾性病例分析方法,总结分析2008年1月~2012年12月收治的8例双侧肾上腺PHEO并PGL病例的临床诊治资料。临床表现为高血压等7例,体检发现肿瘤1例。其中合并多发性内分泌腺瘤(MEN)-2A患者4例。8例患者均检测尿VMA及血、尿儿茶酚胺,尿VMA升高6例(75%),血、尿儿茶酚胺升高5例(62.5%),8例患者均行彩超及CT检查,阳性例数分别为7例(87.5%)及8例(100%)。术前均服用可多华等药物2~4周,分次行腹腔镜两侧PHEO及PGL切除术4例,同期经腹腔入路行腹腔镜双侧PHEO及PGL切除术2例,同期经腹膜后入路行腹腔镜双侧PHEO及PGL切除术2例。结果:PGL位于主动脉旁4例、腔静脉旁2例、肾门区2例。手术均获成功,除1例同期经腹腔入路腹腔镜双侧PHEO及PGL切除术患者术后2周内伴淋巴漏,1例患者双侧同期手术术后出现急性肾上腺皮质功能危象经过及时补充大剂量肾上腺皮质激素以及支持治疗后治愈,其余患者术中、术后未出现明显并发症。瘤体直径3~8cm。术后6~9d出院。随访9个月~4年,7例术前高血压患者术后血压正常6例,仍有高血压1例,未见肿瘤复发。结论:对双侧肾上腺PHEO患者,考虑是否存在MEN;手术时注意可能存在多发肿瘤及同时合并副神经节瘤可能,以避免遗漏;因创伤小、恢复快,应用经腹腔或腹膜后入路同期或分次行双侧腹腔镜PHEO及PGL切除术是可行的;术后需加强长期随访。

Objective:To elucidate the diagnostic and therapeutic procedures of patients with bilateral pheochromocytomas and paraganglioma.Methods:The clinical data of 8patients with bilateral pheochromocytomas and paraganglioma from January 2008 to December 2012 were retrospectively analyzed.Hypertension was observed in 7cases.Another 1case 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 determined in 8cases.The level of urine VMA was elevated in 6cases(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 4cases.Simultaneous operations were performed with laparoscopic surgery via transperitoneal approach in 2cases and via retroperitoneal approach in the rest 2cases.Results:Paragangliomas were localized adjacent to the aorta(4cases),adjacent to cava vein(2cases),and at the renal hilus(2cases).Laparoscopic procedures were successfully performed on all 8cases.No one was converted to open surgery.Lymphatic leakage occurred after surgery in one case and lasted 2weeks.The rest cases had no perioperative and postoperative complications.The diameter of the tumor was from 3to 8cm.The patients were discharged six to nine days postoperatively.During the follow-up period of 9months to 4years,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 operation is necessary.