肾上腺皮质髓质混合性病变的诊治研究

Diagnosis and Management of Mixed Corticomedullary Pathological Changes in Adrenal

目的:提高对肾上腺皮质髓质混合性病变的诊治水平。方法:总结1987～2007年29例肾上腺皮质髓质混合性病变患者的临床资料。临床表现高血压29例,伴头痛16例、心悸10例和肌无力10例。10例血清钾2.4～3.2 mmol/L,平均2.87 mmol/L。血皮质醇9例增高,节律异常2例。24 h尿香草基杏仁酸8例增高。2004年后8例24 h尿甲氧基去甲肾上腺素中1例增高;24 h尿甲氧基肾上腺素中2例增高。24例中血浆肾素:卧位中9例增高,2例降低。立位中7例增高,6例降低。血管紧张素:卧位中7例增高;2例降低。立位中6例增高,4例降低。醛固酮:卧位中5例增高,立位中3例增高。CT扫描检查:7例肾上腺增生,14例肾上腺结节状增生,8例腺瘤,平扫CT值2.6～41.0 Hu,平均21.0 Hu;增强CT值26.0～74.3 Hu,平均57.0 Hu。结果:开放手术8例,后腹腔镜手术21例,手术时间:开放90～180 min,平均110 min;后腹腔镜25～70 min,平均45 min。术中血压变化6例>50 mmHg,23例<50 mmHg。术后7～10天出院。29例中26例随防时间1～5年手术部位未见增生、结节及肿瘤复发。18例患者血压正常,8例仍需服用降压药控制血压,但药物种类和剂量减少。病理诊断:13例肾上腺皮质髓质增生,3例肾上腺皮质髓质增生,皮质增生为主,4例肾上腺皮质髓质增生,髓质增生为主,1例肾上腺皮质髓质增生伴嗜铬细胞瘤,1例肾上腺皮质结节状增生伴髓质增生,3例肾上腺皮质腺瘤伴皮质髓质增生,4例肾上腺皮质腺瘤伴髓质增生。结论:肾上腺皮质髓质混合性病变临床少见,确诊靠病理诊断。手术是治疗的主要方法,腹腔镜下手术值得推荐。

Objective： To study the diagnosis and treatment of mixed corticomedullary pathological changes （MCMPC） in adrenal. Methods：29 eases with MCMPC from 1987 to January 2007 were reviewed. Of 29 patients presented with blood hypertension, 16 in headache, 10 in palpitations, and 10 in paralysis. 10 cases were hypokal emia（2.4-3.2 mmol/L, average 2.87mmol/L）, 9 cases high serum cortisol concentration, 2 cases abnormal rhythm. 8 high 24-h urine VMA. One of 8 cases diagnosised recent 5 years was high in Methoxy norepinephrine, and 2 cases high in 24 h urine Methoxy-adrenergic. 24 cases plasma rennin： 9 cases increased, 2 cases decrease with supine posture; 7 cases increased, 6 cases decrease with standing posture. Angiotension-conversion enzyme： 7 cases increased, 2 cases decrease with supine posture; 6 cases increased, 4 cases decreased with standing posture. Plasma aldosterone concentration：5 cases increased with supine posture, 3 cases increased with standing posture. Of 29 cases underwent CT scan. 7 cases adrenal enlargement, 14 cases adrenal nodule enlargement, and 8 cases adrenal adenoma. Plain CT value of 2.6-41.0 Hu, mean of 21.0 Hu. Enhanced CT value of 26.0-74.3 Hu, with mean of 57.0 Hu. Results： 8 patients underwent open adrenalectomy and 21 patients retroperitoneal laparo-scopic adrenaleetomy. Mean open surgical time was 110 min （range 90-180 min） and mean retroperitoneal laparo seopic time 45 min（range 25-70 min）. Intraoperative changes in blood pressure in 6 cases 〉 50 mmHg, 23 cases 〈50 mmHg. Postoperative hospital stay was 7 to 10 days. 26 cases were no hyperplasia, nodular and tumor recurrence in surgical site durning 1-5 years follow up. 18 cases of blood pressure remained normal, 8 case still need to use antihypertensive agents to control blood pressure, but the type and dose of drugs were reduced. Final pathological diagnosis was as follow： 13 cases in corticalmedullary hyperplasia, 3 cases in corticalmedullary hyperplasia with severe cortical hyperplasia, 4 cases in corticalmedullary hyperplasia with severe medullary hyperplasia, 1 case in corticalmedullary hyperplasia with phechomocytoma, 1 case in cortical nodule hyperplasia, 3 cases in adenoma with corticalmedullary hyperplasia, 4 eases in adenoma with medullary hyperplasia. Conclusions： MCMPC was uncommon in clinic, finally diagnosed in pathological examination. Surgical resection is main option in treatment of MCMPC. laparoscopic surgery was recommended.