球囊辅助下治疗肾血管平滑肌脂肪瘤合并下腔静脉瘤栓一例报告并文献复习

目的报道球囊辅助下治疗肾血管平滑肌脂肪瘤伴下腔静脉瘤栓1例,结合文献复习加深对此病的认识。方法20岁女性患者,术前影像学检查提示右肾巨大肿瘤伴下腔静脉瘤栓,在球囊辅助阻断下行右肾切除及下腔静脉取栓术。结果病理报告为右肾血管平滑肌脂肪瘤,伴下腔静脉瘤栓,长6．5cm,术后随访1年未见复发。结论球囊辅助阻断下腔静脉可用于治疗肾血管平滑肌脂肪瘤伴下腔静脉瘤栓。     

肾血管平滑肌脂肪瘤18例报告

目的 探讨肾血管平滑肌脂肪瘤的诊断及治疗。方法 回顾性分析近10年来收治的肾血管平滑肌脂肪瘤18例的临床资料。结果 18例术前行B超检查．16例行CT检查。手术治疗16例．其中行肾切除术2例．肿瘤剜除术14例，术后均病理检查证实；定期随访2例．随访2～5年，无肿瘤恶变及复发。结论 对肾血管平滑肌脂肪瘤直径大于4cm．可手术治疗；对肿瘤直径小于4cm，可观察。     

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     

肾上皮样血管平滑肌脂肪瘤临床特点分析

目的 探讨肾上皮样血管平滑肌脂肪瘤的临床及病理特点.方法 肾上皮样血管平滑肌脂肪瘤患者6例.单发4例,多发2例.肿瘤直径平均9(5～14)cm.有结节硬化家族史1例.CT检查发现肿瘤内含脂肪组织(CT值-30～-120 HU)3例,诊断为肾血管平滑肌脂肪瘤;3例瘤组织CT值30～55 HU,增强扫描CT值70～110 HU.行肿瘤剜除术3例,行根治性肾切除术3例.结果 3例行肿瘤剜除术者肿瘤完整剜除,2例出现肾盂破损,行肾盂修补及留置双J管.其中1例双肾肿瘤者,2个月后手术剜除对侧肿瘤.3例根治性肾切除者完整切除肾脏、肾周脂肪组织及肿大淋巴结.病理检查:镜下肿瘤以上皮样细胞为主,并出现多形、异形核,有明显的上皮样分化,浸润破坏性生长,瘤组织广泛坏死.免疫组化染色HMB45(+++),EMA、CK(-).病理诊断均为肾上皮样血管平滑肌脂肪瘤.平均随访26(10～44)个月,肿瘤无复发及转移5例;1例根治术后18个月出现双肺转移,10个月后死亡.结论 肾上皮样血管平滑肌脂肪瘤多为良性,部分缺乏脂肪组织,主要由单核或多核的上皮样细胞构成,免疫组化染色HMB45强阳性为特征表现,具有潜在恶性倾向,可发生转移;CT检查易误诊为肾癌;手术为主要治疗方法.     

肾血管平滑肌脂肪瘤破裂出血诊治体会(附20例报告)

目的总结肾血管平滑肌脂肪瘤(renalangiomyolipoma,RAML)出血的诊治经验。方法20例RAML出血患者,14例肿瘤直径>4cm出血难自止者经急诊手术治疗,其中行肾切除8例,肾部分切除和肿瘤剜除术各3例;肿瘤直径<4cm的6例先行抗休克治疗后行肾切除术、肿瘤剜除术各3例。结果术后病理检查均为RAML破裂出血。随访6～36月肿瘤无复发,行保肾手术(肾部分切除、肿瘤剜除术)的9例患肾功能正常。结论B超及CT对RAML出血的诊断具有重要意义,治疗原则常根据肿瘤大小及症状而定。     

肾上腺髓性脂肪瘤5例报告

目的提高对肾上腺髓性脂肪瘤的认识和诊治水平。方法对1992年12月~2003年11月收治的5例肾上腺髓性脂肪瘤患者的临床资料进行回顾性分析。结果4例为右侧偶发瘤,1例出现右腰痛就诊。3例术前行内分泌学检查无异常,4例经CT或MRI均确诊为肾上腺髓性脂肪瘤,1例CT误诊为右肾错构瘤。均行手术切除肿瘤,术后病理报告均为肾上腺髓性脂肪瘤。术后随访0.5~11.5年,均未见肿瘤复发。结论肾上腺髓性脂肪瘤多数无症状,术前通过CT或MRI等影像学技术多能确诊。对瘤体直径<3.5cm的无症状偶发瘤可密切随访,对有症状者或瘤体直径>3.5cm者宜行手术切除。     

腹腔镜下根治性肾切除并肾静脉及腔静脉取栓术

目的 探讨腹腔镜下根治性肾切除并肾静脉及腔静脉取栓术的可行性. 方法 右肾占位病变患者2例.增强CT显示1例肿物部分延伸至肾静脉及腔静脉内,1例右肾静脉内可见充盈缺损并突入腔静脉内.均在全麻下行经后腹腔镜下根治性右肾切除及肾静脉、腔静脉取栓术.术中放置4个穿刺套管针,切断肾动脉后游离腔静脉及肾静脉,腔镜血管阻断钳部分阻断腔静脉,切开腔静脉取出瘤栓,缝合腔静脉,完整切除肾脏及瘤栓. 结果 2例患者的腔静脉瘤栓长度分别为0.3和1.0 cm,均安全取出,术后恢复良好,5 d出院.病理诊断分别为上皮样肾血管平滑肌脂肪瘤和肾透明细胞癌1～2级.术后随访5个月未见肿瘤复发和转移. 结论 对选择性肾肿瘤并肾静脉及腔静脉瘤栓患者行腹腔镜下根治性肾切除并肾静脉及腔静脉取栓术安全可行.     

肾表皮样囊肿1例报告并文献复习

目的探讨肾表皮样囊肿的临床特点、诊断和治疗方法。方法回顾分析1例肾表皮样囊肿伴肾结石患者的临床资料。结果患者行根治性右肾切除,病理报告为右肾表皮样囊肿。患者术后1周痊愈出院,随访10个月未见肿物复发转移。结论肾表皮样囊肿为罕见的肾脏良性肿瘤,临床上报道极少。临床症状缺乏特异性,诊断较困难,需与肾脏肿瘤、囊性肾癌等相鉴别。     

肾血管平滑肌脂肪瘤的保留肾单位手术

目的　评估保留肾单位手术在肾血管平滑肌脂肪瘤治疗中的作用。　方法　回顾性分析 49例行保留肾单位手术治疗的肾血管平滑肌脂肪瘤患者资料 ,观察术前及术后肾功能、手术并发症及预后等。肿瘤剜除术 3 1例 ,肾部分切除 15例 ,原位灌注肾部分切除术 3例 ;其中 3例患者术前行选择性肾动脉栓塞术。　结果　切除肿瘤平均直径 6.0cm (2 .5～ 16.0cm) ,3例原位灌注肾部分切除术肿块直径分别为 10 .5cm、12 .0cm和 15.0cm。 49例术中平均失血量 3 10ml (50～ 10 0 0ml)。无手术并发症发生。术前及术后 10d血肌酐值比较差异无显著性 [(89± 2 6)mmol/Lvs (98± 3 4 )mmol/L ,P>0 .0 5]。 45例平均随访 3 6个月 (1～ 76个月 ) ,无肿瘤复发或需血液透析治疗者。　结论　保留肾单位手术是外科治疗肾血管平滑肌脂肪瘤的首选方法     

肾上皮样血管平滑肌脂肪瘤诊治探讨

目的 提高肾上皮样血管平滑肌脂肪瘤(EAML)的诊治水平.方法 肾EAML患者5例.男3例,女2例.平均年龄46(26～64)岁.患侧腰部胀痛3例,体检偶然发现2例.肿瘤直径2.9～10.1 cm.B超和CT检查诊断为血管平滑肌脂肪瘤2例,肾癌、肾上腺肿瘤、肝癌各1例.行肾肿瘤剜除术3例,肾楔形切除术1例,肾切除术1例.结果 病理报告:肿瘤由不同比例的异常厚壁血管、平滑肌和脂肪组织构成.上皮样细胞胞质浓密嗜酸性或透亮,有嗜酸性颗粒,并有丰富的血管,上皮样细胞簇常围绕血管.组织学形态表现为圆形、多边形和梭形上皮样细胞灶性分布于脂肪瘤中,上皮样瘤细胞免疫组化标记SMA、Vimentin、HMB45表达阳性.5例病理诊断均为肾EAML.随访2～12个月,未见局部复发及远处转移.结果 肾EAML由厚壁血管、脂肪和平滑肌细胞混合组成,上皮样细胞簇常围绕血管.确诊依赖于临床、影像学及病理学检查相结合,免疫组织化学染色有助于诊断及鉴别诊断.早期诊断及完整切除肿瘤是治疗关键.     

Successful interferon-alpha therapy of renal cell carcinoma with lung metastasis: a case report

A 67-year-old man was admitted with a chief complaint of dyspnea. Computed tomography (CT) revealed a left renal tumor, 12 cm in diameter, with tumor thrombus in the left renal vein, multiple lung metastases, right pleural effusion, and para-aortic lymph node metastases. Because of poor condition in respiratory status, nephrectomy was considered to be impossible and the patient was administered 6×10? IU of interferon-alpha daily. Then, 6×10? IU was also administered three times a week. At 14 months after interferon-alpha therapy, CT scan showed complete remission of lung, pleura metastases and right pleural effusion, and the left renal tumor was markedly reduced in size. Because of improvement of general condition, nephrectomy of the left kidney was performed. Twenty-seven months after the start of interferon-alpha therapy, the patient is alive without evidence of disease.     

A case of spontaneous rupture of renal cell carcinoma

A case of spontaneous rupture of renal cell carcinoma is reported. A 53-year old man was admitted with the chief complaint of sudden gross hematuria and right flank pain on December 28, 1979. On the following day, the clinical impression was right ruptured kidney, and therefore right nephrectomy was done. Pathological diagnosis was renal cell carcinoma. He received the post-operative irradiation of a total of 5,000 rads. He was seen five years later, at which time there was no evidence of local recurrence or distant metastasis of cancer. Thirty three cases of spontaneous rupture of renal cell carcinoma were collected from Japanese and English literature. Most common chief complaint is abdominal or flank pain. Excretory urography, ultrasonography, CT scan and angiography are useful, but it is difficult to diagnose preoperatively when the tumor is small. Therefore, it is important to suspect occult cancer when a reasonable cause of rupture is undetermined. In these indeterminate cases primary nephrectomy should be considered strongly.     

结节性硬化症相关肾透明细胞癌一例报告并文献复习

目的 探讨结节性硬化症(tuberous sclerosis complex,TSC)相关肾细胞癌(renal cell carcinoma,RCC)的临床诊治特点.方法 回顾性分析1例TSC相关TCC患者的资料.男,22岁.自幼智障伴癫痫.发现双肾肿瘤5年,间断肉眼血尿2年,曾于1年内2次行超选择性左肾动脉栓塞术,但分别于术后6、10个月再次出现肉眼血尿.查体:智障表现,肥胖,头颈部血管纤维瘤及非创伤性趾甲沟纤维瘤.CT平扫检查左肾有一不规则实性占位,最大截面14.2 cm×9.0 cm,CT值45～54HU,增强扫描病变呈不均匀强化,CT值60～78 HU,左肾静脉内有充盈缺损;右肾见多发脂肪密度占位,CT值-38～-25 HU,最大截面7.2 cm × 5.7 cm.检索Pubmed和CBM数据库进行相关文献复习.结果 患者行经腹开放性左肾根治性切除术,病理诊断肾透明细胞癌,肾静脉内见瘤栓.随访4个月未发现肿瘤复发及转移.结论 TSC相关RCC临床罕见,诊断后可行肾部分切除术或根治性肾切除术.

Abstract：

Objective To explore the diagnosis and treatment features of tuberous sclerosis complex associated renal cell carcinoma.Methods A 22-year-old boy with a childhood history of epilepsy and mental retardation presented with a complaint of intermittent painless gross hematuria for the past 2 years.After superselective left renal artery embolization was done twice in the past year, painless gross hematuria was still repeated with 6- 10 months intervals.Physical examination showed retarded face, obesity, visible facial angiofibroma and a ditch fibroma.CT scan showed irregular lesions.The largest cross-section 14.2 cm × 9.0 cm in the left kidney was inhomogeneous enhanced from 45 - 54 HU in the plain phase to 60 - 78 HU in the contrast phase.Filling defect in the left renal vein and multiple fat-density lesions (CT value of -25 - -38 HU) with the largest cross-section 7.2 cm× 5.7 cm in the right kidney were also found in contrast CT scan.The PUBMED and CBM database were reviewed.Results Open retroperitoneal radical left nephrectomy was performed.Pathology showed renal clear cell carcinoma and renal vein thrombosis.There was no tumor recurrence or distant metastasis at 4-month follow-up.Conclusions Tuberous sclerosis complex associated renal cell carcinoma is rarely reported.Timely nephron-sparing surgery is necessary when the diagnosis is established, or radical nephrectomy is also necessary if nephron-sparing surgery is impossible.     

腹腔镜治疗肾错构瘤并肾静脉及下腔静脉瘤栓1例并文献复习

目的：探讨全腹腔镜治疗肾错构瘤并肾静脉及下腔静脉瘤栓的可行性分析。方法：回顾性分析1例腹腔镜治疗肾错构瘤并肾静脉及下腔静脉瘤栓患者的临床资料。患者,女,26岁,体检时发现右肾占位,B超示右肾窦内可见5．1cm×2．7cm高回声占位,边界欠规则,内见血流。CT示右肾盂旁可见一不规则团块状混杂密度影,大小为4．5cm×2．9cm×1．9cm,可见脂肪成分,最低密度-40HU;病变软组织部分明显强化,增强前后CT值分别为31HU和97HU,病变主要位于肾窦,部分延伸至肾静脉及腔静脉内。检索Pubmed和CBM数据库相关文献进行复习。结果：患者在全麻下行腹腔镜右肾切除及肾静脉、下腔静脉取栓术,瘤栓进入下腔静脉0．6cm。病理诊断右肾错构瘤。术后随访6个月无肿瘤复发和转移。结论：肾错构瘤并。肾静脉及下腔静脉瘤栓临床罕见,对选择性病例行腹腔镜肾切除并行肾静脉及下腔静脉取栓术安全可行。     

肾淋巴管扩张症临床分析

目的 总结肾淋巴管扩张症的临床特点及治疗方法。 方法 分析2008年12月、2010年6月收治的2例肾淋巴管扩张症患者临床资料。例1,女,37岁。右腰腹部痛8d。B超检查示双肾周混合性回声,与肾脏分界欠清。CT检查示双肾被膜下低密度影,内混有散在点状高密度影。保守治疗3周后疼痛缓解。3个月后右侧腰痛加重,CT检查示右肾周被膜下大量积液,B超引导下穿刺置管引流后积液消失;2个月后复查B超示左侧肾周少量积液,穿刺抽液后行积液常规及瘤细胞检查。例2,女,32岁。左腰部酸痛不适3年。B超检查示左肾周围不规则囊性病变,CT检查示左肾后外侧分叶状囊性病变,与肾实质分界不清,左肾受压前移。诊断为左肾淋巴管瘤,硬膜外麻醉下行淋巴管瘤切除术。 结果 例1囊液涂片见大量淋巴细胞和少量中性粒细胞,考虑为淋巴液,临床诊断为肾淋巴管扩张症,随访2个月未见复发。例2术后病理检查囊壁衬以扁平上皮细胞伴淋巴细胞浸润,淋巴管呈囊状扩张,诊断为肾囊性淋巴管瘤。术后随访9个月未见复发。 结论 B超、CT检查有助于肾淋巴管扩张症的诊断,穿刺细胞学和组织病理学检查可确诊。无症状者可密切随访,有症状者可穿刺引流,但复发率较高;也可手术切除扩张淋巴管+无水乙醇破坏内皮细胞治疗,复发率低,但可能发生淋巴漏。     

Extrarenal nephroblastoma: report of a case and review of the literature

A 3-year-old girl with an extrarenal nephroblastoma arising from the right retroperitoneal space is described. She was admitted to our hospital with the chief complaint of abdominal pain. On physical examination, she was found to have a large (12 X 10 cm in size), firm and nontender mass in the right upper quadrant of the abdomen. The mass did not extend beyond the midsagital line. The physical examination did not reveal any particular findings or any congenital anomalies. Urinalysis and hematological data were within the normal limit. Radiological examinations including CT scan showed that the solid tumor was related to the right kidney. Under the diagnosis of right nephroblastoma, 15 micrograms/kg/day of actinomycin D was given intravenously for 5 days from October 18, 1982. The regression rate of the tumor was 78 percent on CT scan after chemotherapy. On November 22, 1982, transperitoneal nephrectomy was performed through a right paramedian incision. The tumor was found to adhere tightly to the upper pole of the kidney. The surgical specimen was 76 g in weight. A section of the surgical specimen showed an extrarenal tumor located completely outside the kidney and separated from the renal cortex by a thickened renal capsule. Histological diagnosis was extrarenal nephroblastoma showing renal capsular invasion by epithelial tumor cells. No teratomatous components were encountered in the tumor.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of bilateral renal cell carcinoma

A case of asynchronous bilateral renal cell carcinoma is reported. A 52-year-old man with the chief complaint of asymptomatic gross hematuria visited our hospital on November 16, 1981. Intravesicular pyelography showed poor secretion of the left kidney, compression of its pelvis and calyces and normal visualization of the right kidney. On computer tomographic (CT) scanning, abdominal aortogram and left selective renal angiogram, a round tumor lesion on the left kidney was recognized. Under the diagnosis of left renal tumor, radical left nephrectomy was performed in December, 1981. Histological diagnosis was clear cell carcinoma of the left kidney. He was administered medroxyprogesterone acetate. In December, 1985, CT showed a space-occupying lesion laterally on the right kidney and another suspected tumorous lesion. On the right selective renal angiogram, a round hypervascular lesion about 3 cm in diameter was found on the upper portion of the right kidney. On February 10, 1986 simple surgical enucleation of the 2 renal tumors was performed. Histological diagnosis was the same as that of the left kidney. Postoperative course was uneventful. He is well without recurrence or metastasis 8 months after operation.     

A case of primitive neuroectodermal tumor of the kidney]

A primitive neuroectodermal tumor (PNET) is a solid tumor originating from the neural crest. This tumor is known to occur in the central nervous system and soft tissue, but recently determined to also invade the kidney. Although primary renal PNET is very rare, we encountered a case of primary renal PNET with multiple lung metastasis. This case was a 35-year-old man with a chief complaint of macroscopic hematuria, which he noticed in January 1996. He was hospitalized because of right renal tumor detected with ultrasonography. On CT scan, a low-density solid tumor with a maximum diameter of 8 cm was visualized in the right kidney. Low-signal and high-signal tumorous lesions were demonstrated in the same region on T 1-weighted and T 2-weighted MR images, respectively. Then right radical nephrectomy was performed on Feb. 5, 1996. On histopathological observation, the tumor was composed of small tumor cells with solid growth. Immunohistochemical staining revealed that the tumor was positive for CD 99. Thus our final diagnosis was primary right renal PNET. Although 2 metastatic lesions with a diameter of about 1 cm were observed in the right lower lung before the time of surgery, pulmonary metastatic lesions markedly increased in number 3 months after surgery. Thus we initiated CAP chemotherapy with cyclophosphamide, doxorubicin, and CDDP. After 3 cycles, pulmonary metastatic lesions disappeared at CT scan, indicating complete remission. However, a tumor with a maximum diameter of about 10 cm recurred in the retroperitoneum 7 months after complete remission. Although 2 cycles of CAV/PE therapy with cyclophosphamide, doxorubicin, vincristine, CDDP, and VP-16 were performed following resection of the retroperitoneal tumor, he showed no response and decreased 24 months after surgery.     

A case of bilateral renal cell carcinoma

A 69-year-old man visited our clinic with the chief complaint of macroscopic hematuria. On CT scanning and renal arteriography, a round tumor about 3 cm in diameter on the frontlateral phase of the left kidney and another tumor ranging from the upper pole to the center of the right kidney were recognized. Under the diagnosis of bilateral renal tumor, first in November 1980 left partial nephrectomy was performed to extirpate the tumor. Then, upon recovery from postoperative transient renal hypofunction, right nephrectomy was performed in January 1981. After the operation renal hypofunction was noted again, but in March of the same year he was discharged as his creatinine value was stabilized to 3 approximately 4 mg/dl. Histopathologically the tumor of the left kidney was clear cell subtype and that of the right kidney was granular cell subtype of renal cell carcinoma. He has been followed up under administration of PSK and CQ in the outpatient clinic. As of January, 1985 he is well without recurrence or metastasis.