1、遗传性肾癌综合征张洪宪文档ppt意义意义早期筛查早期筛查仔细随访相应患者及其家属仔细随访相应患者及其家属降低疾病相关死亡率并改善预后降低疾病相关死亡率并改善预后发现遗传性肾癌的相关基因发现遗传性肾癌的相关基因进行肿瘤形成机制相关的细胞分子通路研究进行肿瘤形成机制相关的细胞分子通路研究进行肿瘤治疗的分子靶点的研究进行肿瘤治疗的分子靶点的研究种类种类 VHLVHL综合征综合征遗传性乳头状肾细胞癌遗传性乳头状肾细胞癌(HPRC)(HPRC)遗传性平滑肌瘤病及肾细胞癌综合征遗传性平滑肌瘤病及肾细胞癌综合征(HLRCC)(HLRCC)BirtBirtHoggHoggDube Dube 综合征综合征结节性
2、硬化病结节性硬化病(TS)(TS)？2022-11-264Von HippleVon HippleLindau Lindau 综合征综合征 1895 1895 年由德国眼科教授年由德国眼科教授Eugen von HippleEugen von Hipple首先发现首先发现19261926年由瑞典病理学家年由瑞典病理学家Avid LindauAvid Lindau再次确认再次确认19361936年由年由DavisonDavison教授总结相关临床表现并命名为教授总结相关临床表现并命名为von von Hippel-Lindau syndromeHippel-Lindau syndrome 是一种
3、相对罕见的常染色体显性遗传病，发病率是一种相对罕见的常染色体显性遗传病，发病率1/360001/36000主要表现包括肾透明细胞癌，嗜铬细胞瘤，视网膜成血管主要表现包括肾透明细胞癌，嗜铬细胞瘤，视网膜成血管母细胞瘤，中枢神经系统成血管细胞瘤等母细胞瘤，中枢神经系统成血管细胞瘤等基因学研究基因学研究 VHL VHL 基因定位在常染色体基因定位在常染色体3p26-253p26-25目前已被完全测序，并确认是存在于散发性和家目前已被完全测序，并确认是存在于散发性和家族性肾透明细胞癌中的抑癌基因族性肾透明细胞癌中的抑癌基因该基因的丢失、突变和甲基化失活导致正常的该基因的丢失、突变和甲基化失活导致正常的
4、VHLVHL蛋白合成障碍，是导致蛋白合成障碍，是导致VHLVHL综合征的重要分综合征的重要分子学基础子学基础 2022-11-267BirtHoggDube syndrome遗传性乳头状肾细胞癌(HPRC)VHL综合征诊断标准More recently,detailed histologic description has led to more refined characterization of the pathologic features now termed HLRCC renal tumorsThe familial association of perifollicular d
5、ermatosis involving the face and trunk among three first-degree relatives was first described by Hornstein and Knickenberg in 1975fumarate hydratase,a Krebs cycle enzyme既往体健，否认高血压病史，否认家族史，体格检查血压轻度升高150/78mmHg遗传性平滑肌瘤病及肾细胞癌肾癌病理类型基本是透明细胞癌亚型，肿瘤体积越小倾向恶性程度越低The gene product,folliculin,is thought to be inv
6、olved in regulation of the mammalian target of rapamycin(mTOR)pathway by acting through folliculin-interacting protein(FNIP-1)and 50-AMP-activated protein kinase体积较小的肾脏肿瘤（3cm）恶性度低但长期服用免疫抑制剂是否增加VHL综合征其他系统肿瘤的发病率？1936年由Davison教授总结相关临床表现并命名为von Hippel-Lindau syndrome肾脏其他病理类型肿瘤未发现VHL基因突变chromophobe嫌色细胞,o
7、ncocytoma,clear cell and hybrid oncocytic tumors composed of elements of oncocytoma and chromophobe但长期服用免疫抑制剂是否增加VHL综合征其他系统肿瘤的发病率？potentially misclassified as collecting duct tumors结合上述临床表现诊断为VHL综合征。Two years later,Drs Birt,Hogg and Dube described clinical dermatologic findings involving 15 family m
8、embers with similar skin nodules described as fibrofolliculomas纤维毛囊瘤,trichodiscomas毛盘状瘤 acrochordons软垂疣该基因在该基因在VHLVHL病家族成员中突变率几乎达病家族成员中突变率几乎达100%100%散发的肾透明细胞癌患者中，散发的肾透明细胞癌患者中，VHLVHL基因突变率为基因突变率为46%46%70%70%肾脏其他病理类型肿瘤未发现肾脏其他病理类型肿瘤未发现VHLVHL基因突变基因突变 VHLVHL综合征临床表现综合征临床表现2022-11-269VHLVHL综合征诊断标准综合征诊断标准 （1
9、 1）中枢神经系统或视网膜成血管细胞瘤家族病史，）中枢神经系统或视网膜成血管细胞瘤家族病史，有一种成血管细胞瘤或内脏病变（如肾肿瘤、胰腺肿有一种成血管细胞瘤或内脏病变（如肾肿瘤、胰腺肿瘤或囊肿、嗜铬细胞瘤、附睾乳头状囊腺瘤等）瘤或囊肿、嗜铬细胞瘤、附睾乳头状囊腺瘤等）（2 2）对于无明确家族遗传史的孤立病例，若患有两）对于无明确家族遗传史的孤立病例，若患有两种或两种以上成血管母细胞瘤种或两种以上成血管母细胞瘤,或一种成血管母细胞或一种成血管母细胞瘤和一种内脏病变瘤和一种内脏病变 类型类型 I I型不表现为肾上腺嗜铬细胞瘤，病变可累及中枢神经型不表现为肾上腺嗜铬细胞瘤，病变可累及中枢神经系统、肾
10、脏、胰腺等系统、肾脏、胰腺等型伴发肾上腺嗜铬细胞瘤型伴发肾上腺嗜铬细胞瘤AA型，不伴有肾癌型，不伴有肾癌BB型，伴有肾癌型，伴有肾癌CC型，仅有肾上腺嗜铬细胞瘤表现型，仅有肾上腺嗜铬细胞瘤表现 临床特点临床特点一般情况下，病变是视网膜成血管母细胞瘤最早出一般情况下，病变是视网膜成血管母细胞瘤最早出现，然后是中枢神经系统血管母细胞瘤，而肾癌出现，然后是中枢神经系统血管母细胞瘤，而肾癌出现较晚现较晚中枢神经系统血管母细胞瘤和肾脏透明细胞癌为该中枢神经系统血管母细胞瘤和肾脏透明细胞癌为该病最常见的致死原因病最常见的致死原因嗜铬细胞瘤，临床上多因出现高血压症状而发现嗜铬细胞瘤，临床上多因出现高血压症状
11、而发现其他病变如胰腺囊肿、附睾或阔韧带乳头状囊腺瘤、其他病变如胰腺囊肿、附睾或阔韧带乳头状囊腺瘤、肾囊肿肾囊肿,多无明显症状，一般预后良好多无明显症状，一般预后良好 VHLVHL综合征是遗传性肾癌最常见的原因综合征是遗传性肾癌最常见的原因肾癌也是肾癌也是VHLVHL综合征主要的恶性肿瘤综合征主要的恶性肿瘤与散发的肾癌相比并无特异性与散发的肾癌相比并无特异性 肾癌占肾癌占VHLVHL综合征患者死亡原因的综合征患者死亡原因的50%50%，发生率为，发生率为24247 70%0%加上肾囊肿，加上肾囊肿，VHLVHL综合征患者中肾脏病变的发生率可达到综合征患者中肾脏病变的发生率可达到60%60%肾脏病
12、变的平均年龄为肾脏病变的平均年龄为3939岁岁(16(1667)67)体积较小的肾脏肿瘤（体积较小的肾脏肿瘤（3cm3cm）恶性度低）恶性度低VHLVHL综合征肾脏病变为多灶性综合征肾脏病变为多灶性 WaltherWalther等对等对VHLVHL综合征患者的肾脏标本进行研究，综合征患者的肾脏标本进行研究，显微镜下观察，发现有的标本中存在显微镜下观察，发现有的标本中存在600600个肿瘤病个肿瘤病灶和灶和11001100个囊肿病灶个囊肿病灶随访研究表明由单纯囊肿变为肾癌的可能性很小随访研究表明由单纯囊肿变为肾癌的可能性很小所以所以VHLVHL综合征的单纯肾囊肿若没有症状一般无综合征的单纯肾囊肿
13、若没有症状一般无需特殊治疗需特殊治疗 但是复杂的囊肿有可能包含肿瘤组织而逐渐生长，但是复杂的囊肿有可能包含肿瘤组织而逐渐生长，需要定期监测需要定期监测 散发的肾癌一样，散发的肾癌一样，VHLVHL综合征肾癌缺乏早期临床综合征肾癌缺乏早期临床症状，通常在很长时间内都没有任何表现症状，通常在很长时间内都没有任何表现 肾癌进展的病例可以表现为血尿，疼痛或肿块肾癌进展的病例可以表现为血尿，疼痛或肿块 肾癌病理类型基本是透明细胞癌亚型，肿瘤体积肾癌病理类型基本是透明细胞癌亚型，肿瘤体积越小倾向恶性程度越低越小倾向恶性程度越低与非与非VHLVHL综合征肾癌相比，综合征肾癌相比，VHLVHL综合征肾癌的发综
14、合征肾癌的发病年龄较早，通常表现为双侧多中心的实性和囊病年龄较早，通常表现为双侧多中心的实性和囊性的病变性的病变 治疗治疗VHLVHL综合征患者肾癌的预后与肿瘤的大小密切相关综合征患者肾癌的预后与肿瘤的大小密切相关 对最大径超过对最大径超过3cm3cm的肿瘤行肾部分切除术，这样可以的肿瘤行肾部分切除术，这样可以减低肿瘤的转移的风险而且保留肾脏的功能减低肿瘤的转移的风险而且保留肾脏的功能对于直径较小的肿瘤（对于直径较小的肿瘤（3cm3cm）可以选择密切观察）可以选择密切观察 一项研究对肾脏肿瘤小于一项研究对肾脏肿瘤小于3cm3cm的的108108例例VHLVHL综合征患者与肿综合征患者与肿瘤大于
15、瘤大于3cm3cm的的7373例患者做比较例患者做比较中位时间超过中位时间超过5 5年随访结果显示肿瘤小于年随访结果显示肿瘤小于3cm3cm患者中无病例患者中无病例发生转移，而肿瘤大于发生转移，而肿瘤大于3cm3cm组组7373例患者中有例患者中有2020例发生转移例发生转移（27%27%）VHLVHL综合征肾癌常为双侧多发，肿瘤生长较慢，转移较晚，综合征肾癌常为双侧多发，肿瘤生长较慢，转移较晚，肾脏肿瘤平均每年增长肾脏肿瘤平均每年增长一般不建议对一般不建议对VHLVHL综合征行肾根治性切除术，即使为单侧综合征行肾根治性切除术，即使为单侧肾癌，也应尽量行保留肾单位的肿瘤切除手术，因为对侧肾癌，
16、也应尽量行保留肾单位的肿瘤切除手术，因为对侧肾脏也有再发生肾癌的可能肾脏也有再发生肾癌的可能 如果无法保留肾脏，可选择进行双侧肾根治性切除术，如果无法保留肾脏，可选择进行双侧肾根治性切除术，再透析或行肾移植术加服免疫抑制剂再透析或行肾移植术加服免疫抑制剂当当VHLVHL患者接受肾移植以后，移植肾无发展为肾囊肿患者接受肾移植以后，移植肾无发展为肾囊肿或肾癌的倾向或肾癌的倾向但长期服用免疫抑制剂是否增加但长期服用免疫抑制剂是否增加VHLVHL综合征其他系统综合征其他系统肿瘤的发病率？肿瘤的发病率？VHLVHL基因抑癌机制清楚，抑癌作用明显，而且基因抑癌机制清楚，抑癌作用明显，而且VHLVHL基基因
17、只有因只有3 3个外显子，是基因治疗十分理想的目的基因个外显子，是基因治疗十分理想的目的基因目前目前VHLVHL基因治疗还处在体外研究动物实验阶段基因治疗还处在体外研究动物实验阶段VHLVHL基因治疗将为基因治疗将为VHLVHL综合征治疗开辟一个新的方向综合征治疗开辟一个新的方向 随访随访VHLVHL综合征合并肾癌的患者应每年复查一次综合征合并肾癌的患者应每年复查一次CTCT或或MRIMRI如果最大肿瘤直径超过如果最大肿瘤直径超过3cm3cm，就应对所有肿瘤行，就应对所有肿瘤行肿瘤剜除术或肾部分切除术肿瘤剜除术或肾部分切除术有有VHLVHL家族病史的人，也应该每年复查一次家族病史的人，也应该每
18、年复查一次CTCT对于无肿瘤的单纯囊肿，不推荐手术切除对于无肿瘤的单纯囊肿，不推荐手术切除 遗传性乳头状肾细胞癌遗传性乳头状肾细胞癌指的是病人易于罹患肾乳头状细胞癌的状态指的是病人易于罹患肾乳头状细胞癌的状态与与c-Metc-Met原癌基因的突变有关，定位于染色体原癌基因的突变有关，定位于染色体7q317q31常染色体显性遗传常染色体显性遗传临床表现临床表现发病隐匿，多无明显临床表现发病隐匿，多无明显临床表现多为多灶性，双侧发病多为多灶性，双侧发病影像学表现为乏血供的肿瘤，影像学表现为乏血供的肿瘤，CTCT增强仅表现为轻度强增强仅表现为轻度强化（增加化（增加10-30HU10-30HU），），
19、MRIMRI增强仅增强仅15%15%2022-11-2624散发的肾透明细胞癌患者中，VHL基因突变率为46%70%遗传性平滑肌瘤病及肾细胞癌Renal cancers with a prevalence estimated between 2 and 21%散发的肾透明细胞癌患者中，VHL基因突变率为46%70%结合上述临床表现诊断为VHL综合征。中枢神经系统血管母细胞瘤和肾脏透明细胞癌为该病最常见的致死原因但长期服用免疫抑制剂是否增加VHL综合征其他系统肿瘤的发病率？患者手术采用平卧位略垫高腰部，取双侧肋缘下人字形切口至两侧腋中线。Mean age at diagnosis is olde
20、r,near age 50 yearsrenal tumorspulmonary metastases from a case of locally advanced clear cell carcinoma in a 20-year-old patient遗传性平滑肌瘤病及肾细胞癌加上肾囊肿，VHL综合征患者中肾脏病变的发生率可达到60%患者实施双肾部分切除术，左肾上腺切除术。主要表现包括肾透明细胞癌，嗜铬细胞瘤，视网膜成血管母细胞瘤，中枢神经系统成血管细胞瘤等治疗治疗通常选择肾部分切除术通常选择肾部分切除术术中仔细检查，防止遗漏病灶术中仔细检查，防止遗漏病灶遗传性平滑肌瘤病及肾细胞癌A r
21、elatively new,rare and aggressive form of HRC A relatively new,rare and aggressive form of HRC syndromesyndromecutaneous leiomyomascutaneous leiomyomas uterine leiomyomas uterine leiomyomas renal cell carcinoma renal cell carcinomafumarate hydratase,a Krebs cycle enzymefumarate hydratase,a Krebs cyc
22、le enzymeGeneticsGeneticsBe mapped to a region on chromosome 1(1q42.3-Be mapped to a region on chromosome 1(1q42.3-43)43)encodes for the HLRCC gene product,fumarate encodes for the HLRCC gene product,fumarate hydratasehydratasean autosomal dominant patternan autosomal dominant patternthe tumor suppr
23、essor function of the genethe tumor suppressor function of the geneClinical featuresClinical featuresthe finding of severely symptomatic uterine the finding of severely symptomatic uterine fibroids among affected women within familiesfibroids among affected women within familiesoften requiring early
24、 hysterectomy due to often requiring early hysterectomy due to difficulties from menometrorrhagiadifficulties from menometrorrhagia89%of affected women underwent hysterectomy89%of affected women underwent hysterectomy44%before the age of 3044%before the age of 30Isolated cases of uterine leiomyosarc
25、omasIsolated cases of uterine leiomyosarcomasCutaneous leiomyomas are common among affected Cutaneous leiomyomas are common among affected individuals,though may be difficult to identifyindividuals,though may be difficult to identifyRenal cancers with a prevalence estimated Renal cancers with a prev
26、alence estimated between 2 and 21%between 2 and 21%papillary type 2 tumors papillary type 2 tumors potentially misclassified as collecting duct tumors potentially misclassified as collecting duct tumorsMore recently,detailed histologic description has More recently,detailed histologic description ha
27、s led to more refined characterization of the led to more refined characterization of the pathologic features now termed HLRCC renal pathologic features now termed HLRCC renal tumorstumorsManagementManagementRadiographic appearance of HLRCC tumors may appear Radiographic appearance of HLRCC tumors m
28、ay appear partly cystic and poorly definedpartly cystic and poorly definedNephron-sparing surgery is less well established in this Nephron-sparing surgery is less well established in this setting setting Surgical intervention must be performed with care to Surgical intervention must be performed wit
29、h care to ensure minimal handling of the tumor and complete wide ensure minimal handling of the tumor and complete wide resection,including lymph node dissectionresection,including lymph node dissectionPreoperative PET scans may prove beneficial in cases in Preoperative PET scans may prove beneficia
30、l in cases in which lymph node or nonlocalized disease is suspectedwhich lymph node or nonlocalized disease is suspectedBirtBirtHoggHoggDube syndromeDube syndromeThe familial association of perifollicular dermatosis The familial association of perifollicular dermatosis involving the face and trunk a
31、mong three first-degree involving the face and trunk among three first-degree relatives was first described by Hornstein and Knickenberg relatives was first described by Hornstein and Knickenberg in 1975in 1975Two years later,Drs Birt,Hogg and Dube described clinical Two years later,Drs Birt,Hogg an
32、d Dube described clinical dermatologic findings involving 15 family members with dermatologic findings involving 15 family members with similar skin nodules described as fibrofolliculomassimilar skin nodules described as fibrofolliculomas纤维毛囊纤维毛囊瘤瘤,trichodiscomas,trichodiscomas毛盘状瘤毛盘状瘤 acrochordons
33、acrochordons软垂疣软垂疣GeneticsGeneticsautosomal dominant patterns of inheritance autosomal dominant patterns of inheritance chromosomechromosomeThe gene product,folliculin,is thought to be involved The gene product,folliculin,is thought to be involved in regulation of the mammalian target of rapamycin i
34、n regulation of the mammalian target of rapamycin(mTOR)pathway by acting through folliculin-(mTOR)pathway by acting through folliculin-interacting protein(FNIP-1)and 50-AMP-activated interacting protein(FNIP-1)and 50-AMP-activated protein kinaseprotein kinaseClinical featuresClinical featuresClassic
35、 triad of skin fibrofolliculomasClassic triad of skin fibrofolliculomas pulmonary cysts pulmonary cysts renal tumors renal tumorsrenal tumors were discovered in 14-34%.renal tumors were discovered in 14-34%.Spontaneous pneumothoraces occurred in Spontaneous pneumothoraces occurred in 23%:most common
36、 in younger family members 23%:most common in younger family members(40 years)(40 years)Lung cysts were common and seen in 83%Lung cysts were common and seen in 83%Skin lesions in 90%Skin lesions in 90%the relative heterogeneity of tumor subtypes the relative heterogeneity of tumor subtypes indolent
37、 forms of diseaseindolent forms of diseasechromophobechromophobe嫌色细胞嫌色细胞,oncocytoma,clear cell,oncocytoma,clear cell and hybrid oncocytic tumors composed of and hybrid oncocytic tumors composed of elements of oncocytoma and chromophobeelements of oncocytoma and chromophobeMean age at diagnosis is ol
38、der,near age 50 yearsMean age at diagnosis is older,near age 50 yearsPathologic analysis of 130 tumors obtained from 30 Pathologic analysis of 130 tumors obtained from 30 surgically managed cases identified proportional surgically managed cases identified proportional differences including hybrid on
39、co/chromo 50%,differences including hybrid onco/chromo 50%,chromophobe 34%,conventional clear cell 9%,chromophobe 34%,conventional clear cell 9%,oncocytoma 5%and papillary 2%oncocytoma 5%and papillary 2%less aggressive tumor histologiesless aggressive tumor histologiesnot be considered an indolent d
40、isease processnot be considered an indolent disease processaggressive tumor features in young patientsaggressive tumor features in young patients pulmonary metastases from a case of locally advanced pulmonary metastases from a case of locally advanced clear cell carcinoma in a 20-year-old patientcle
41、ar cell carcinoma in a 20-year-old patientOne 39-year old patient with BHD and mixed renal tumor,One 39-year old patient with BHD and mixed renal tumor,including clear cell components developed distant including clear cell components developed distant progression and deathprogression and death2022-1
42、1-26392022-11-26402022-11-26412022-11-2642ManagementManagementintervention for tumors,which grow to reach 3cm in intervention for tumors,which grow to reach 3cm in size and include the use of nephron-sparing size and include the use of nephron-sparing procedures when possibleprocedures when possible
43、总结总结中、青年居多，有中、青年居多，有/无家族史无家族史肾肿瘤多为双侧、多发肾肿瘤多为双侧、多发合并其它脏器病变合并其它脏器病变染色体和基因异常染色体和基因异常治疗：肾肿瘤直径小于治疗：肾肿瘤直径小于3cm3cm者观察等待，当肾肿瘤直者观察等待，当肾肿瘤直径大于径大于3cm3cm时可手术，以时可手术，以NSSNSS首选首选病例病例男性，男性，2121岁岁体检体检B B超发现双肾占位，胰腺占位超发现双肾占位，胰腺占位3 3周就诊周就诊患者无明显的症状，无血尿、心悸、出汗、头痛。患者无明显的症状，无血尿、心悸、出汗、头痛。既往体健，否认高血压病史，否认家族史，体格既往体健，否认高血压病史，否认家
44、族史，体格检查血压轻度升高检查血压轻度升高150/78mmHg150/78mmHg腹部腹部CTCT显示双肾多个大小不等低密度影，部分边缘欠清晰，显示双肾多个大小不等低密度影，部分边缘欠清晰，增强后，右肾上极及左肾门处见不均匀性强化，分别为，。增强后，右肾上极及左肾门处见不均匀性强化，分别为，。内未见脂肪密度。双肾内亦可见多个不强化低密度影，最内未见脂肪密度。双肾内亦可见多个不强化低密度影，最大者于右肾实质内，大小，增强后边缘较前清晰。左肾上大者于右肾实质内，大小，增强后边缘较前清晰。左肾上腺区类圆形软组织肿块影，密度均匀，边缘光滑，大小，腺区类圆形软组织肿块影，密度均匀，边缘光滑，大小，增强后
45、见明显均匀强化。胰颈部、尾部见斑片状低密度影，增强后见明显均匀强化。胰颈部、尾部见斑片状低密度影，增强后有轻度强化。增强后有轻度强化。CTCT影像表现提示双肾多发肿瘤、多发影像表现提示双肾多发肿瘤、多发囊肿，左肾上腺肿瘤，胰腺囊腺瘤囊肿，左肾上腺肿瘤，胰腺囊腺瘤2022-11-2646头颅头颅MRIMRI显示左侧延髓见类圆形混杂信号灶，大显示左侧延髓见类圆形混杂信号灶，大小约，增强扫描明显强化，符合血管母细胞瘤小约，增强扫描明显强化，符合血管母细胞瘤行眼底荧光血管造影提示双眼视网膜大动脉血管行眼底荧光血管造影提示双眼视网膜大动脉血管母细胞瘤母细胞瘤阴囊阴囊B B超提示双侧附睾头囊肿，左侧，右侧
46、，精液超提示双侧附睾头囊肿，左侧，右侧，精液囊肿可能囊肿可能结合上述临床表现诊断为结合上述临床表现诊断为VHLVHL综合征。术前检查综合征。术前检查患者血清儿茶酚胺水平位于正常范围内患者血清儿茶酚胺水平位于正常范围内 患者实施双肾部分切除术，左肾上腺切除术。胰腺病变考患者实施双肾部分切除术，左肾上腺切除术。胰腺病变考虑良性肿瘤可能性大，另外同期手术创伤大，故未一期同虑良性肿瘤可能性大，另外同期手术创伤大，故未一期同时处理时处理患者手术采用平卧位略垫高腰部，取双侧肋缘下人字形切患者手术采用平卧位略垫高腰部，取双侧肋缘下人字形切口至两侧腋中线。手术先处理右肾。术中可见右肾表面有口至两侧腋中线。手术
47、先处理右肾。术中可见右肾表面有多发大小不等的透明囊肿，囊肿直径最大约，用电刀将多多发大小不等的透明囊肿，囊肿直径最大约，用电刀将多发囊肿逐一去顶。同时肾表面可见发囊肿逐一去顶。同时肾表面可见8 8个淡黄色结节样质硬个淡黄色结节样质硬肿瘤，肿瘤直径最大约，位于肾下极背侧近肾门处，突出肿瘤，肿瘤直径最大约，位于肾下极背侧近肾门处，突出肾脏表面，遂用剪刀将直径六处结节样肿瘤距瘤体周缘处肾脏表面，遂用剪刀将直径六处结节样肿瘤距瘤体周缘处完整切除肿瘤，用完整切除肿瘤，用3-03-0可吸收线将其间断缝合，部分中间可吸收线将其间断缝合，部分中间填塞止血纱布填塞止血纱布阻断右肾动脉，将另外两处大肿瘤同法切除，
48、中间留置自阻断右肾动脉，将另外两处大肿瘤同法切除，中间留置自体腰部所取肌肉组织、止血纱布，体腰部所取肌肉组织、止血纱布，2-02-0肝针线贯穿间断缝肝针线贯穿间断缝合肾脏伤口处合肾脏伤口处2323针，止血满意，动脉阻断时间针，止血满意，动脉阻断时间12min12min，同法处理左肾多发囊肿，左肾表面淡黄色结节样质硬肿瘤同法处理左肾多发囊肿，左肾表面淡黄色结节样质硬肿瘤共共1616处，不阻断动脉切除其中处，不阻断动脉切除其中1515块大小不等肿物，然后阻块大小不等肿物，然后阻断肾动脉，切除位于左肾上极背侧近肾门处，直径约断肾动脉，切除位于左肾上极背侧近肾门处，直径约3cm3cm的肿瘤，动脉阻断时间的肿瘤，动脉阻断时间7min7min。左肾上腺肿物直径约为。左肾上腺肿物直径约为2cm2cm，位于左肾上腺下极，完整切除肿瘤及部分肾上腺位于左肾上腺下极，完整切除肿瘤及部分肾上腺
