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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 investigate the diagnosis and treatment of bilateral adrenal pheochromocytoma (PHEO) and paraganglioma (PGL). Methods: A retrospective case analysis method was used to summarize the clinical data of 8 cases of bilateral adrenal PHEO and PGL admitted from January 2008 to December 2012. Clinical manifestations of hypertension in 7 cases, physical examination found 1 case of tumor. Among them, 4 cases were complicated with multiple endocrine neoplasia (MEN) -2A. All 8 patients were tested for urinary VMA, blood and urinary catecholamines, urinary VMA in 6 cases (75%) and blood and urinary catecholamines in 5 cases (62.5%). All 8 cases were examined by color Doppler and computed tomography (CT) They were 7 cases (87.5%) and 8 cases (100%) respectively. Preoperative medication may be more Chinese and other drugs 2 to 4 weeks, sub-divided into two laparoscopic PHEO and PGL resection in 4 cases, the same period of laparoscopic bilateral laparoscopic PHEO and PGL resection in 2 cases, the same period by the peritoneum Two patients underwent laparoscopic bilateral PHEO and PGL resection. Results: PGL was located in the aorta in 4 cases, vena cava in 2 cases and in the portal area in 2 cases. Surgery was successful, except for 1 case of simultaneous laparoscopic bilateral PHEO and PGL resection by laparoscopic surgery within 2 weeks after surgery with lymphatic leak, 1 case of bilateral simultaneous surgery after acute adrenal crisis occurred after timely Additional high-dose adrenocorticotropic hormone and supportive treatment after treatment, the remaining patients during operation, no significant postoperative complications. Tumor diameter 3 ~ 8cm. 6 ~ 9d after discharge. Follow-up 9 months to 4 years, 7 cases of preoperative hypertension in patients with normal blood pressure in 6 cases, there is still 1 case of hypertension, no tumor recurrence. CONCLUSIONS: In patients with bilateral adrenal PHEO, the presence or absence of MEN is considered; attention may be given to the possibility of multiple tumors and concurrent paraganglioma at the time of surgery in order to avoid omission; due to small trauma and rapid recovery, the application of transabdominal or retroperitoneal approach Or sub-line bilateral laparoscopic PHEO and PGL resection is feasible; need to strengthen long-term follow-up.