非典型肾球旁细胞瘤5例临床分析及手术治疗效果观察

Clinical analysis and operation outcome of atypical juxtaglomerular cell tumors in 5cases

目的观察非典型肾球旁细胞瘤患者临床特征,探讨手术治疗的效果和安全性。方法回顾性分析5例非典型肾球旁细胞瘤患者的临床资料。5例术前行全腹部CT平扫+增强扫描及胸部CT平扫检查,测定血钾水平,行立卧位醛固酮试验测定卧位及立位时血浆肾素活性、血管紧张素Ⅱ和醛固酮水平。3例腹部CT提示肾上腺增粗者测定促肾上腺皮质激素、血皮质醇节律、肾上腺素、去甲肾上腺素、多巴胺及尿香草苦杏仁酸水平,其中2例提示肾上腺髓质增生。5例于全身麻醉下行机器人辅助腹腔镜或传统腹腔镜辅助保留肾单位手术,2例肾上腺髓质增生者同期行肾上腺切除术。记录手术情况,术后漏尿、肾动静脉瘘、感染及延迟出血等并发症发生情况;手术切除肿瘤组织常规行组织病理及免疫组织化学检查。术后每6~12个月复查CT,测定血浆肾素活性、血管紧张素Ⅱ和醛固酮水平及血压;随访6~92个月,记录肿瘤复发、转移及血压、肾素活性、醛固酮、血钾等情况。结果5例术前腹部CT平扫均表现为肾内类圆形低密度病灶,增强扫描示病灶呈实性软组织密度影,边界清,形态规则,延迟期、髓质期轻中度强化,3例CT检查提示肾上腺增粗;5例胸部CT检查均未见异常。5例术前血钾水平[(3.59~5.07)mmol/L]正常;卧位血浆肾素活性为9.64~24.71ng/(mL·h)、血管紧张素Ⅱ为93.90~126.00ng/L、醛固酮为146.40~352.00ng/L,立位血浆肾素活性为14.50~25.30ng/(mL·h)、血管紧张素Ⅱ为103.00~155.30ng/L、醛固酮为187.20~609.00ng/L,均升高;3例腹部CT提示肾上腺增粗者促肾上腺皮质激素、血皮质醇节律、肾上腺素、去甲肾上腺素及多巴胺水平均正常,其中2例尿香草苦杏仁酸水平升高,分别为86、89μmol/24h。5例手术均顺利完成,手术时间90~126 min,术中出血量40~100mL,术后均未发生漏尿、肾动静脉瘘、感染及延迟出血等并发症。5例术后组织病理均证实为肾脏球旁细胞瘤,镜下可见纤维包膜,与周围肾组织分界清晰;肿瘤间质血管丰富,肿瘤细胞呈中等大小,细胞核形态规则,位于胞质中央,未见病理性核分裂;免疫组织化学示CD34、Vimentin阳性,CK阴性,Ki-67阳性表达率5%~15%,4例SMA阳性,4例HBM-45阴性。随访至2022年4月,5例血压及血浆肾素活性、血管紧张素Ⅱ、醛固酮水平均正常,未发生肿瘤复发或转移。结论非典型肾球旁细胞瘤患者临床可表现为高血压、高肾素活性、高醛固酮水平,血钾水平正常;行保留肾单位手术或联合肾上腺切除术治疗可降低患者高血压,安全性好。

Objective To observe the clinical features of atypical juxtaglomerular cell tumors,and to investigate the efficacy and safety of operation.Methods The clinical data of 5patients with atypical juxtaglomerular cell tumors were retrospectively analyzed.All patients underwent plain and enhanced abdominal CT and plain chest CT before operation,and were measured the blood potassium levels.The response of aldosterone to upright posture was evaluated to detect the plasma renin,angiotensinⅡand aldosterone at supine and standing positions.Three patients with CT confirmed adrenal hypertrophy were detected the corticotropic hormone,blood cortisol rhythm,epinephrine,norepinephrine,dopamine and urinary vanillyl mandelic acid levels,showing adrenal medulla hyperplasia in 2patients.Five patients were performed Robot assisted or conventional laparoscopic nephron-sparing surgery,in which 2patients with combined adrenal medulla hyperplasia were performed simultaneous adrenalectomy.The operation process,postoperative urine leakage,renal arteriovenous fistula,infection,delayed bleeding and other complications were recorded.Routine histopathological and immunohistochemical examinations were performed on the resected tumor tissue.Every 6to 12months after operation,these patients received CT scan,and were detected the plasma renin,angiotensinⅡ,aldosterone levels and blood pressure.During the follow-up survey for 6 to 92 months,the plasma renin,aldosterone levels,blood pressure,and tumor recurrence and metastasis were recorded.Results Preoperative plain CT scans of 5patients showed quasi-circular low density lesions in the kidney.Enhanced CT showed solid soft tissue density,clear borders,regular shapes,delayed and mild to moderate enhancement in medullary phase.Three patients were detected with adrenal enlargement during CT scan.No abnormalities were found by chest CT.The preoperative serum potassium levels in all patients were normal[(3.59-5.07)mmol/L].At supine position,the plasma renin level was from 9.64to 24.71ng/(mL·h),angiotensinⅡwas from 93.90to 126.00ng/L,and aldosterone concentration was from 146.40to 352.00ng/L.At standing position,the plasma renin level was from 14.50to 25.30ng/(mL·h),angiotensinⅡwas from 103.00to 155.30ng/L,and aldosterone concentration was from2ᦆ187.20to 609.00ng/L,higher than those at supine position.Three patients with CT scan confirmed adrenal hypertrophy had normal corticotropic hormone,blood cortisol rhythm,epinephrine,norepinephrine and dopamine,in which 2patients had elevated urinary vanillyl mandelic acid level(86,89μmol/24h).All patients underwent operation successfully in 90to 126min,and the intraoperative blood loss was 40to 100mL.No postoperative urine leakage,renal arteriovenous fistula,infection,or delayed bleeding occurred.The postoperative histopathological examination certified renal juxtaglomeral cell tumors in all patients.The fibrous capsule was visible under the microscope,which was clearly demarcated from the surrounding renal tissue.The tumor interstitium was rich in blood vessels,and the tumor cells were of medium size.The shape of the nucleus was regular,located in the center of the cytoplasm,without pathological mitosis.Immunohistochemical examination showed positive CD34and Vimentin,negative CK,and Ki-67positive rate of 5%to 15%.Four patients were positive SMA,and 4were negative HBM-45.The patients were followed up till April,2022,showing normal blood pressure,plasma renin,angiotensinⅡand aldosterone levels,and no tumor recurrence or metastasis.Conclusions The patients with atypical renal juxtaglomerular cell tumors are clinically featured by hypertension,high renin,high aldosterone,and normal blood potassium level.Nephron-sparing surgery or combined with adrenalectomy can reduce blood pressure and is safe.