保留皮质的肾上腺切除术治疗双侧嗜铬细胞瘤的临床疗效

Analysis of the therapeutic effect of cortical-sparing adrenalectomy on bilateral pheochromocytoma

目的:探讨保留皮质的肾上腺切除术(CSA)治疗双侧嗜铬细胞瘤的疗效和安全性。方法:回顾性分析2004年1月至2019年12月中南大学湘雅医院收治的20例双侧嗜铬细胞瘤患者的临床资料,男、女各10例。平均发病年龄32.5(8~51)岁。3例有嗜铬细胞瘤家族史。双侧同步发病、异时发病患者分别为14例、6例。20例术前尿香草扁桃酸(VMA)平均值为(106.4±60.0)μmol/24h。术前增强CT检查示肾上腺区不均匀强化的软组织肿块,肿块内部呈低密度坏死灶,考虑嗜铬细胞瘤。20例均全麻下行CSA。14例双侧同步发病者中,9例行同期手术,5例行分期手术;6例异时发病者均先后切除双侧肿瘤。腹腔镜手术18例,开放手术2例。经腹或腹膜后途径,打开肾上极周围脂肪囊,游离肾上极内侧缘,显露肾上腺及肿瘤,完整切除肿瘤及包膜,保证肾上腺组织距肿瘤切缘3~5 mm,保留的皮质大小至少为同侧肾上腺的1/3。术中尽量保留肾上腺中央静脉,减少对肾上腺血管床的破坏。记录手术相关数据、术中监护记录、术后相关并发症及远期随访结果。结果:本组20例手术均顺利完成,术中无肿瘤破裂。同期手术和分期手术的手术时间分别为(242.3±61.0)min和(137.9±60.3)min,术后入住ICU例数分别为7例和2例,差异均有统计学意义(P<0.05);术中出血量分别为(528.6±355.7)ml和(277.8±264.7)ml,输血例数分别为5例和2例,术后平均住院时间分别为(7.4±2.0)d和(7.8±3.3)d,差异均无统计学意义(P>0.05)。20例术后口服糖皮质激素(泼尼松5 mg,每12小时1次),无明显肾上腺皮质功能低下表现及艾迪生病危象,于术后2周至1个月逐步停用激素。平均随访5.4(1.0~16.0)年,3例复发,无转移病例。术后对10例进行基因检测,7例携带嗜铬细胞瘤RET、VHL致病基因突变(RET 2例,VHL 5例)。结论:CSA治疗双侧嗜铬细胞瘤尽管具有一定的复发风险,但避免了激素替代且不会增加转移与死亡风险,可在遗传性嗜铬细胞瘤尤其是双侧嗜铬细胞瘤的治疗中探索应用。

Objective To investigate the efficacy and safety of cortical-sparing adrenalectomy(CSA)in the treatment of bilateral pheochromocytoma.Methods The clinical data of 20 patients with bilateral pheochromocytoma treated in Xiangya Hospital of Central South University from January 2004 to December 2019 were analyzed retrospectively,including 10 males and 10 females.The average age of onset was 32.5(8-51)years.3 cases had a family history of pheochromocytoma.There were 14 and 6 patients with bilateral synchronous and metachronous onset,respectively.The mean value of vanilmandelic acid(VMA)in 20 cases was(106.4±60.0)μmol/24h.Preoperative enhanced CT showed a soft tissue mass with uneven enhancement in the adrenal region,with low-density necrosis,which suggested the diagnosis of Pheochromocytoma.All 20 cases underwent CSA under general anesthesia.In 14 cases of bilateral synchronous disease,9 cases underwent simultaneous operation and 5 cases underwent staged operation;6 patients with metachronous disease underwent bilateral tumor resection successively.Laparoscopic surgery was performed in 18 cases and open surgery in 2 cases.Through the abdominal or retroperitoneal approach,open the fat capsule around the upper pole of the kidney,free the medial edge of the upper pole of the kidney,expose the adrenal gland and tumor,completely remove the tumor and capsule,ensure that the adrenal tissue is 3-5 mm away from the cutting edge of the tumor,and the reserved cortical size is at least 1/3 of the ipsilateral adrenal gland.The central adrenal vein was preserved as much as possible to reduce the damage to the adrenal vascular bed.The operation related data,intraoperative monitoring records,postoperative complications and long-term follow-up results were recorded.Results All the 20 cases were successfully completed without tumor rupture.The operation time of simultaneous operation and staged operation were(242.3±61.0)min and(137.9±60.3)min,respectively.The number of patients admitted to ICU after operation was 7 and 2,respectively(P<0.05);The intraoperative bleeding volume was(528.6±355.7)ml and(277.8±264.7)ml,the number of blood transfusion cases were 5 and 2 cases,and the average hospital stay was(7.4±2.0)d and(7.8±3.3)d,respectively(P>0.05).20 cases took glucocorticoid orally(prednisone 5 mg,once every 12 hours)after operation.There was no obvious manifestation of adrenocortical dysfunction and Addison's crisis.The hormone was stopped gradually from 2 weeks to 1 month after operation.The average follow-up was 5.4(1.0-16.0)years.There were 3 cases of recurrence and no metastasis.Gene detection was performed in 10 cases after operation,and 7 cases carried pheochromocytoma RET and VHL pathogenic gene mutations(RET in 2 cases and VHL in 5 cases).Conclusion Although CSA has a certain risk of recurrence,it avoids hormone replacement and does not increase the risk of metastasis and death.It is recommended for the treatment of hereditary pheochromocytoma,especially bilateral pheochromocytoma.