von Hippel-Lindau综合征外科治疗

目的 探讨von Hippel-Lindau(VHL)综合征的外科治疗方法.方法 VHL综合征患者4例.例1,男,56岁.主诉乏力、心悸2 d.空腹血糖2.37 mmol/L.CT检查示左肾上、下极3个肿块,直径分别为8.0、7.0、4.0 cm.10年前行脑血管母细胞瘤切除术.例2,女,57岁.主诉左腰痛不适1个月.CT检查示左肾上腺、左肾、胰体肿物,直径分别为2.7、4.5、2.1 cm.例3,女,39岁.查体发现左肾上腺占位1个月.CT检查示左肾上腺3.0 cm×4.0 cm实性占位,增强后肿块明显强化.既往有小脑、脊髓血管母细胞瘤及双侧肾癌手术史.例4,女,41岁.B超发现双肾肿瘤1个月入院.CT检查示左肾、左肾上腺、右肾、胰腺肿物,直径分别为4.0、3.0、1.5、2.0 cm.1个月前行y刀治疗多发脑部肿瘤.结果 4例均手术治疗.例1行左肾根治性切除术,病理报告肾血管周细胞瘤,随访6个月右肾未见异常.例2行左肾、左肾上腺、胰体尾及脾切除,病理报告肾透明细胞癌、胰岛细胞瘤、肾上腺囊肿,随访3个月MRI检查发现脊髓肿瘤.例3行左肾上腺肿瘤摘除术,病理报告嗜铬细胞瘤,随访2年未见肿瘤复发.例4行左肾、左肾上腺切除,病理报告肾透明细胞癌、肾上腺嗜铬细胞瘤,3周后剜除右肾肿瘤,病理报告透明细胞癌,随访1年肿瘤未复发.结论 VHL可并发多器官肿瘤,较大中枢神经系统肿瘤可手术切除,多发较小的实性肿瘤可放疗.直径<3cm肾癌可密切随访、择期处理;直径>3cm肾癌首选保留肾单位手术.肾上腺肿瘤首选腺瘤摘除术,避免肾上腺全切术.腹部多器官肿瘤一期手术安全可行. 理报告肾透明细胞癌、胰岛细胞瘤、肾上腺囊肿,随访3个月MRI检查发现脊髓肿瘤.例3行左肾上腺肿瘤摘除术,病理报告嗜铬细胞瘤,随访2年未见肿瘤复发.例4行左肾、左肾上腺 除,病理报告肾透明细胞癌、肾上腺嗜铬细胞瘤,3周后剜除右肾肿瘤,病理报告透明细胞癌,随访1年肿瘤未复发.结论 VHL可并发多器官肿瘤,较大中枢神经系统肿瘤可手术切除,多发较小的实性肿瘤可放疗.直径<3cm肾癌可密切随访、择期处理;直径>3cm肾癌首选保留肾单位手术.肾上腺肿瘤首选腺瘤摘除术,避免肾上腺全切术.腹部多器官肿瘤一期手术安全可行. 理报告肾透明细胞癌、胰岛细胞瘤、肾上腺囊肿,随访3个月MRI检查发现脊髓肿瘤.例3行左肾上腺肿瘤摘除术,病理报告嗜铬细胞瘤,随访2年未见肿瘤复发.例4行左肾、左肾上腺 除,病理报告肾透明细胞癌、肾上腺嗜铬细胞瘤,3周后剜除右肾肿瘤,病理报告透明细胞癌,随访1年肿瘤未复发.结论 VHL可并发多器官肿瘤,较大中枢神经系统肿瘤可手术切除,多发较小的实性肿瘤可放疗.直径<3cm肾癌可密切随访、择期处理;直径>3cm肾癌首选保留肾单位手术.肾上腺肿瘤首选腺瘤摘     

肾球旁细胞瘤6例临床分析及文献复习

目的探讨肾球旁细胞瘤的临床特点,提高对该病的诊断和治疗水平。方法回顾性分析6例肾球旁细胞瘤患者临床资料并复习相关文献。男5例,女1例,初诊年龄[26.2±5.7(18～37)]岁,就诊时高血压6例,血浆肾素活性[基础(6479.6±2349.3)ng·L-1·h-1,激发(8446.3±1926.4)ng·L-1·h-1]、血管紧张素Ⅱ[基础(114.8±34.2)pg/ml,激发(297.3±87.1)pg/ml]及血浆醛固酮[基础(0.35±0.04)nmol/L,激发(0.63±0.18)nmol/L]水平增高6例,低血钾4例。除1例拒行CT检查外,5例由动态增强CT定位。结果保守治疗1例,根治性患肾切除1例,保留肾单位的肾肿瘤切除4例,病理组织学及免疫组化证实肾球旁细胞瘤的诊断。术后随访4～160个月,血压、血钾、肾素、醛固酮均在正常范围内,未见肿瘤复发和转移。结论肾球旁细胞瘤具有高血压、高肾素血症、高醛固酮血症、低血钾的临床特点,动态CT增强扫描有助于定位,免疫组化染色CD34、肌动蛋白(Actin)阳性,细胞角蛋白(CK)阴性是其特征,保留肾单位的肿瘤切除术是有效的治疗方法。     

肾球旁细胞瘤1例并文献复习

目的 探讨肾球旁细胞瘤的特点,提高对该病的诊疗水平.方法 回顾性分析1例肾球旁细胞瘤患者临床资料并复习相关文献.该患者为37岁女性,因高血压就诊,体检发现右肾肿瘤,血钾正常,术前诊断为右肾癌而行根治性右肾切除术.结果 术后病理学及免疫组化证实为肾球旁细胞瘤;术后随访3个月,血压、血钾均正常.结论 肾球旁细胞瘤具有高血压、低血钾的特点,但临床上也存在不典型的病例,加强对该疾病的认识可提高术前诊断率及帮助确定恰当的治疗方案;手术是治疗该病的有效手段.     

肾球旁细胞瘤4例报道并文献复习

目的探讨肾球旁细胞瘤的症状、影像学表现、诊断、病理、治疗及预后。方法通过回顾分析4例肾球旁细胞瘤(JGCT)的临床资料,结合相关文献进行讨论。结果 4例患者平均年龄25.5岁,术前均为高血压。3例入院前为低血钾、高肾素、高醛固酮血症。CT呈低密度影,增强扫描可见强化,强化程度低于肾实质。MRI提示稍长T1、T2信号,边界欠清,增强后动脉期及静脉期持续不均匀强化,延迟期减低。分别行经腹腹腔镜下肾肿瘤剜除术、肾癌根治术和机器人辅助腹腔镜下肾肿瘤剜除术。肿瘤组织呈片、巢状排列,漩涡状生长。免疫组化:肿瘤细胞表达CD34(+++),SMA(散在+),HMB45(±),Syn(弱+),ki67(约4%+)。术后患者中位随访时间9个月,均未见高血压、低血钾及肿瘤复发。结论 JGCT临床罕见,术前多有高血压及低血钾症状,CT和磁共振对诊断有帮助,但确诊需组织病理结合免疫组化,可予肿瘤剜除或肾脏切除,预后良好,但极少数存在恶性潜质,需要长期随访。     

肾素瘤的诊断和治疗(附3例报告)

目的提高肾素瘤的诊治和治疗水平。方法回顾分析3例肾素瘤患者的临床资料,结合文献复习进行讨论。3例患者均表现有高血压、高肾素和高醛固酮血症。2例临床表现有低血钾。结果2例患者行保留肾单位的肾肿瘤切除术,1例行肾切除术,术后病理示:肾素瘤。3例患者术后血压、血浆肾素、醛固酮和血钾等恢复正常,随访3～12年未见肿瘤复发及高血压再发。结论肾素瘤为罕见的良性肾脏肿瘤,肿瘤根治性切除效果满意,预后良好。     

肾脏异位肾上腺皮质腺瘤1例报告并文献复习

目的:提高对肾脏异位肾上腺皮质腺瘤的认识和诊疗能力。方法:对收治的1例肾脏异位肾上腺皮质腺瘤患者的诊治经过进行回顾性分析，总结临床经验，并结合文献进行复习归纳，分析术前误诊原因。结果:患者因“恶心、纳差4 d”入院，术前影像学检查提示肾上腺占位，血醛固酮升高，血钾降低，考虑肾上腺醛固酮瘤，术中见肿瘤来源肾脏上极，与肾上腺分界清楚，行3D腹腔镜右肾部分切除手术，术后病理报告(右肾肿物)皮质腺瘤。经过4个月的术后随访，患者血醛固酮、血钾恢复正常，无并发症。结论:肾脏异位肾上腺皮质腺瘤罕见，最终需要手术及病理确诊。肾脏上极异位肾上腺肿瘤或增生，容易误诊，发现后应进行手术确诊，尽可能采用肾部分切除术。     

肾嗜酸细胞瘤二例报告

肾嗜酸细胞瘤是一种非常少见的肾脏良性肿瘤 ,我院收治 2例 ,现报告如下。例 1,男 ,4 5岁 ,查体发现右肾肿块 1周 ,无血尿 ,无尿急、尿痛、发热等表现。查体未发现阳性体征。B超示右肾中部背侧实质内 1.6cm× 1.7cm低回声团块 ,透声差。CT示右肾中部一外突性肿瘤 ,约 1.5cm× 1.2cm ,CT值与肾实质相同。诊断 :右肾占位考虑为肾癌或肾嗜酸细胞瘤。行右肾探查术 ,术中见肿瘤突出于肾轮廓之外 ,外向性生长 ,切取标本送快速病理 ,报告为肾嗜酸细胞瘤。遂行包括肿瘤周围 2cm正常肾组织的肿瘤剜除术 ,手术顺利 ,术后患者恢复好。随访 2年 ,患者…     

肾平滑肌瘤伴同侧嗜酸性细胞瘤1例报告

本文报道1例女性患者, 2020年3月4日因体检发现右肾占位4 d入院。彩色多普勒超声检查示右肾单发占位。腹部增强CT及三维重建检查示右肾2个占位性病变。行后腹腔镜右侧肾部分切除术, 术中确认右肾最下极和中下级各有1个肿物。病理诊断:肾平滑肌瘤、肾嗜酸细胞瘤。患者术后恢复良好, 未行放化疗。术后随访1年, 未见肿瘤复发和转移。单侧肾脏同时发生两种肿瘤性疾病罕见。     

肾嗜酸细胞瘤诊治体会（附5例报告并文献复习）

目的：探讨肾嗜酸细胞瘤早期临床诊断及治疗方法。方法：5例肾嗜酸细胞瘤患者中3例术前诊断为肾癌行肾癌根治术;1例术中冰冻切片证实诊断后行肿瘤剜除术;1例巨大肾占位术前影像学诊断为恶性间皮瘤或脂肪肉瘤,行胸腹联合切口肿瘤切除术。结果：术后随访7～34月,平均15.2月,均无瘤生存。结论：超声、CT及MRI在肾嗜酸细胞瘤的诊断中具有重要作用,对于可疑病例,须行术中快速冰冻病理检查,确诊需要免疫组化检查,宜行保留肾单位手术。     

肾球旁细胞器瘤的临床分型与诊治探讨

目的 介绍一种新的肾球旁细胞器瘤临床分型方法 ,并对其临床诊治进行探讨. 方法 收集肾球旁细胞器瘤患者5例,结合PubMed搜索到较大的系列病例报道66例,对71例病例资料进行分型.对患者的临床表现、实验室检查和影像学检查进行分析.临床资料包括血压;实验室检查包括血钾、血浆肾素和醛固酮、静脉分段取血检测;影像学检查包括超声、CT、IVU、选择性肾血管造影等. 结果 依据血压和血钾水平,71例肾球旁细胞器瘤可分为3种类型:典型、非典型和静止型.57例典型病例特点:高血压、低血钾、高醛固酮和低肾素;12例非典型病例的特点:高血压、正常血钾;2例静止型病例特点:正常血压和血钾.对于具有高血压、低血钾、高肾素和高醛固酮的患者,应考虑典型肾球旁细胞器瘤的可能;对于高血压合并肾肿瘤的患者,应考虑非典型球旁细胞器瘤的可能;对于单纯肾脏肿瘤,应考虑静止型肾球旁细胞器瘤的可能. 结论 肾球旁细胞器瘤划分为典型、非典型和静止型3类的依据是血压和血钾.肾球旁细胞器瘤代表了一种可以手术纠正的高血压病变,肾肿瘤鉴别诊断中应考虑肾球旁细胞器瘤的可能.保留肾单位手术是首选治疗方法 .     

Juxtaglomerular cell tumor of the kidney—a new classification scheme

ObjectiveTo introduce a new classification scheme of juxtaglomerular cell tumor (JCT) of the kidney for differential diagnosis of hypertension and renal cell carcinoma.MethodsFive cases of JCT have been diagnosed and treated surgically in our hospital during the last 4 years. Through a search in PubMed, we incorporated 7 large series of case reports of JCT into a review of 71 cases previously published in the literature. Clinical presentations (blood pressure), laboratory examinations [serum potassium, plasma renin activity (PRA), aldosterone (ALD), and renal venous sampling for renin assay], and imaging examinations [ultrasonography, computerized tomography (CT), excretory urography, and selective renal angiography] were summarized.ResultsThe 71 cases of JCTs can be classified into 3 types, which are typical type, atypical type, and non-functioning type. The 57 typical cases had the typical characteristics of hypertension, hypokalemia, hyperaldosteronism, and high renin. The 12 atypical cases had hypertension with normal potassium levels, and the 2 non-functioning cases had normal blood pressure and normal potassium levels.ConclusionsThe classification of typical, atypical, and non-functioning JCTs depends on blood pressure and serum potassium. JCT of the kidney should be considered in patients with hypertension and renal tumor, and nephron-sparing surgery is the first choice.     

Renal transplantation unveils Conn's syndrome: a case report

Primary hyperaldosteronism or Conn's syndrome is rare after renal transplantation. We present a case of a 34-year-old woman with end-stage renal disease, who had received a living renal transplant and showed persistent hypertension and hypokalemia. The common causes of hypertension after kidney transplantation were excluded and hypokalemia persisted despite potassium supplementation and angiotensin converting enzyme inhibitors. Biochemical findings revealed extremely elevated plasma aldosteron levels, suppressed plasma renin activity, and massive urinary potassium wasting. Abdominal computerized tomography scan showed a left 25-mm adrenal nodule. Her hypertension and hypokalemia was cured by the removal of the adenoma.     

Renin Secreting Tumors: Diagnosis, Conservative Surgical Approach and Long-Term Results

During the last 15 years 8 patients were diagnosed with renin secreting juxtaglomerular cell tumors among 30,000 hypertensive patients. Clinical characteristics included severe hypertension poorly medically controlled in young patients (mean age 22.3 years) and severe hypokalemia (mean 2.83 mmol./l.). Secondary hyperaldosteronism was present in all cases with a constant elevation of renin activity. Renal vein sampling was only positive in 64 percent of cases. Selective renal arteriography demonstrated an avascular area in 43 percent of the patients. Computerized tomography showed the tumor in all cases. Mean tumor size was 24 mm. (range 10 to 50). Conservative surgery was feasible in all patients. Perioperative ultrasonography was used for 3 intraparenchymal tumors. Hypertension and hypokalemia resolved within 1 week after surgery. At a mean followup of 98 months (range 24 to 204) no tumor recurrence was documented.     

Surgical treatment of primary hyperaldosteronism.

OBJECTIVE: A retrospective review of patients with primary hyperaldosteronism treated at the Duke University Medical Center was performed. SUMMARY BACKGROUND DATA: The management of patients with primary hyperaldosteronism has changed dramatically in the past 20 years. The outcome of surgical treatment was examined to optimize the management of these patients. METHODS: All patients who were evaluated for primary hyperaldosteronism before operation and who had adrenal surgery at Duke University Medical Center from 1970 to 1993 were included in the study. RESULTS: A ratio of plasma aldosterone concentration to plasma renin activity greater than 250 was predictive of hyperaldosteronism in 94% of patients. This ratio also predicted the size of the adenoma. Surgical treatment cured hypokalemia in 90% of the patients. Hypertension was cured or greatly improved, with an average decrease of 50.4 mmHg and 25.2 mmHg for systolic and diastolic pressures, respectively. Patients who had hypertension for less than 5 years achieved a satisfactory blood pressure response more often (13 of 14 patients) than did patients who had hypertension for 5 or more years (15 of 20 patients). CONCLUSIONS: Preoperative evaluation of patients with primary hyperaldosteronism using current modes allows a posterior approach to be used in most patients. Adrenalectomy cures hypokalemia and hypertension; however, patients with a history of hypertension for less than 5 years may be more likely to be cured by adrenal resection.     

Laparoscopic resection of a juxtaglomerular cell tumor of the right kidney

Juxtaglomerular cell tumor is an extremely rare neoplasm of the kidney that causes blood hypertension. A 45-year-old man with persistent hypertension was referred to our department because of a solid mass of unclear origin (kidney? colon?) located in the right mesorenal region that had been detected by Computed tomography (CT) scan, ultrasonography, and colonoscopy. Serum levels of renin, plasma renin activity (PRA), and aldosterone were all normal. Operatively, four 10/12-mm ports were placed, ultrasonography confirmed a well-encapsulated lesion of the kidney. Ultrasonic shears and cautery were used to resect the lesion, including 5 mm of free renal tissue. Postoperatively, a prompt normalization of blood pressure was observed. The pathological findings showed a juxtaglomerular cell tumor. The definitive diagnosis of juxtaglomerular cell tumor is made on the basis of laboratory findings, and imaging studies, Its definitive treatment is surgical excision which can be accomplished successfully with minimally invasive surgery.     

A case report of juxtaglomerular cell tumor incidently found with a tumor hemorrhage

A 17-year-old man was admitted to our hospital with a chief complaint of right flank pain. Both computed tomography and magnetic resonance imaging demonstrated a tumor hemorrhage in a mass located at the upper pole of the right kidney. He had a high blood pressure of 220/100 mmHg associated with high serum renin activity (36.2 ng/ml/hr). Partial nephrectomy was performed and histopathological examination revealed a juxtaglomerular cell tumor. Postoperatively, his blood pressure was markedly improved and the serum renin activity immediately fell into the normal range. Previously, 32 cases of juxtaglomerular cell tumor have been reported in the Japanese literature, and to our knowledge, this is the first case was found with a tumor hemorrhage.     

Juxtaglomerular cell tumor of the kidney treated with nephron-sparing surgery

A case of juxtaglomerular cell tumor of the right kidney is reported. A 30-year-old woman visited us with a complaint of headaches. Severe hypertension and an elevated level of plasma renin activity was seen at the initial evaluation. Computerized tomographic angiography revealed tumor vessels in the low-density mass in the right kidney. The preoperative diagnosis was renin-secreting tumor of the kidney, and nephron-sparing surgery was performed. The pathological findings showed a juxtaglomerular cell tumor. Postoperatively, prompt normalization of blood pressure and a reduced plasma renin activity level were observed.     

Hyperreninemia and secondary hyperaldosteronism in a patient with pheochromocytoma and von Hippel-Lindau disease.

In a 21-year-old Caucasian women with von Hippel-Lindau disease, norepinephrine-producing adrenal pheochromocytoma was identified as the underlying cause of severe hypertension. She was found to have extremely elevated levels of circulating renin and aldosterone, and she was markedly hypokalemic. Administration of captopril further enhanced renin secretion, while her blood pressure improved. The patient became normokalemic following tumor removal, and her blood pressure decreased to normal levels with reestablishment of normal circadian blood pressure rhythm. This case demonstrates that, in the absence of renovascular or malignant hypertension, pheochromocytoma can be the underlying cause for the clinical syndrome of hypertension associated with severe hypokalemia and hyperreninemic hyperaldosteronism.