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1、成 人 肾 母 细 胞 瘤(Adult Wilms Tumor)病 例患者青年女性,35岁,已婚病历号:02126450主诉“间断性左侧腰部疼痛1周”现病史:一周前无明显诱因出现左侧腰部疼痛,呈绞痛,伴恶心、呕吐,为胃内容物,无血尿,无尿频、尿急、尿痛,无腹胀、腹泻,自服去痛片不能缓解。在家休息一夜后症状缓解,在外院查CT示左肾占位,逐来我院。病 例既往史:无特殊体格检查:生命体征平稳,心肺腹查体未见明显异常。双肾区无异常隆起无叩击痛,双肾下极可触及,双侧输尿管走行区无压痛,耻骨上区无压痛,膀胱未充盈,正常女性外阴,尿道外口无红肿,无分泌物。病 例化验检查1.血、尿常规:未见异常。 2.生化全
2、项:肝功能正常,血肌酐及尿素氮正常。病 例辅助检查心电图,胸片未见异常。泌尿系B超(2007-02-26,本院):左肾中下极肾盂旁探及一类圆形直径3.1cm低回声实性占位,外形规则,表面光滑,内部回声不均,未见血流信号,肾静脉及下腔静脉未见瘤栓,肾门及腹膜后未见肿大淋巴结。结论:左肾CaT1N0V0MX病例CT(2007-02-14,外院):左肾体积略增大,左肾后下段可见一类圆形肿块影,包膜完整,大小约4.3cm4cm4cm,平扫密度不均匀,病变内散在类圆形及新月形低密度影CT值19-28Hu,周边可见花边样较高密度影,CT值41-58Hu。增强扫描低密度区未见明显强化,较高密度区可见轻度至中
3、度强化,CT值66-86Hu,强化程度明显低于肾实质,周围肾实质明显受压变薄,左侧肾窦受压,肾窦内局部肾血管及肾盂、肾盏受压移位。结论:左肾占位性病变,合并局部出血。 病 例病 例病例入院后于2007-02-27行左肾根治性切除术:1.术中见肿瘤位于左肾中段,部分压迫肾盂,包膜完整,外周呈黄色,中心呈暗黑色。2.术后病理示(病检号:07-0293):(左)肾母细胞瘤(Wilms瘤),上皮型。属中度危险肿瘤。肿瘤大小:4cm 3.5cm 3cm。可见大片出血、坏死。肿瘤以上皮性成分为主,多为早期上皮性分化。未分化的胚芽成分较少见。未见明显间变。肿瘤局限于肾内,期。手术断端未见肿瘤。免疫组化:AE
4、1/AE3(部分+),VIM(部分+), NSE(部分+), WT1(部分+)。 回顾肾母细胞瘤( nephroblastoma) 又称威尔姆斯瘤(Wilms tumor ,WT) ,是婴幼儿最常见的恶性实体瘤之一,80 %以上患者小于5岁,发病平均年龄为3.5 岁。临床表现主要是腰腹部包块(超过90%),其他还有肉眼血尿,发烧,腹痛,高血压,腹水等。少部分患儿伴有先天畸形,如泌尿生殖系畸形(Genitourinary anomalies) 、半身肥大(hemihypertrophy) 、 Denys-Drash 综合征、WAGR 综合征、Beckwith-Wiedemann 综合征,或同时患
5、其他恶性肿瘤。*成人诊断标准Kilton 于1980 年提出了成人肾母细胞瘤的诊断标准: (1) 肾脏原发性肿瘤;(2) 具有原始母细胞样梭形或圆形细胞成分;(3) 病理发现不成熟胚胎样肾小球、肾小管样结构;(4) 无肾癌组织;(5) 组织学图像明确;(6)年龄大于15 岁。Kilton LKilton L,Matthews MJMatthews MJ,Cohen MHCohen MH:Adult WilmsAdult WilmsAdult WilmsAdult Wilms tumor: A report of prolonged survival and tumor: A report of
6、 prolonged survival and tumor: A report of prolonged survival and tumor: A report of prolonged survival and review of the literature.review of the literature.review of the literature.review of the literature. J Urol 1980;124:1-5 J Urol 1980;124:1-5基因表达WilmsWilms Tumor in Adults: Aspiration Cytology
7、and Cytogenetics Tumor in Adults: Aspiration Cytology and CytogeneticsPeng Li, M.D., Ph.D., Mary Ann Perle, Ph.D., John V. Scholes, M.D., and Grace C.H. Yang, M.D.Department of Pathology, New York University School of Medicine, New York, New YorkDiagnostic Cytopathology, Vol 26, No 2临床表现症状多为腹痛、血尿、季肋
8、部包块。最常见转移部位为肺、肝、脑、骨。临床分期期期:肿瘤局限于肾脏能被完整切除;肿瘤没有侵透肾脏被膜,没有累及肾窦血管期期:肿瘤超出肾脏仍能被完整切除;无淋巴结转移;至少有下列一项:侵透肾脏被膜,侵及肾窦血管,术前有穿刺活检史,术中肿瘤局限性破裂期期:术后有大体或微小残存包括: 不能切除的肿瘤,切缘阳性,肿瘤破裂污染腹膜,区域淋巴结转移,脉管瘤栓期期:血行转移(肺、肝、骨、脑) 或腹部以外的淋巴结转移期期: 双侧肾母细胞瘤。Kalapurakal JA , Dome JS ,Perlman EJ ,et al .Kalapurakal JA , Dome JS ,Perlman EJ ,et
9、 al .Management of WilmsManagement of WilmsManagement of WilmsManagement of Wilmstumor : current practice tumor : current practice tumor : current practice tumor : current practice and future goals.and future goals.and future goals.and future goals. Lancet Oncol ,2004 , 5 :37246. Lancet Oncol ,2004
10、, 5 :37246.辅助检查B超:仅能发现肿块,而不能提示肿块的性质。CT(成人肾母细胞瘤): 1. CT 平扫表现为实性肾肿块,来自肾皮质,一般较大,在肾内膨胀性或弥漫性生长,亦有向外生长者。肿物的密度明显低于正常的肾实质。有时可以发现肿物内部的密度不均匀,可能为肿物内部出血、坏死或者钙化所致。 2. CT 增强扫描时可以发现,肿物呈不均匀强化,其内部可以有出血区、坏死区。肿块与肾实质之间也可以出现线状强化的假包膜影1。1.1.FRENBACK S K. FRENBACK S K. Nephroblastomotosis :comparison of CT and urography J
11、.Nephroblastomotosis :comparison of CT and urography J .Nephroblastomotosis :comparison of CT and urography J .Nephroblastomotosis :comparison of CT and urography J . Radiology ,1998 Radiology ,1998 ,166 :153 - 155.,166 :153 - 155.辅助检查肾动脉造影(成人肾母细胞瘤):表现为肿瘤呈少血性,供养血管纤细,排列整齐,呈弯曲波浪状进入肿瘤组织,即“蔓藤状血管”。82 %的成
12、人肾母细胞瘤在肾动脉造影上具有上述特点,所以可以以此作为与其他肾脏肿瘤相鉴别的依据1。而典型肾癌在肾动脉造影上表现为肿瘤内血管丰富,可见动静脉瘘,并有肿瘤染色等。1.1.Kioumehr F , Cochran S T , Layfield L , et al.Kioumehr F , Cochran S T , Layfield L , et al. Wilms tumor (nephroblastoma) in the Wilms tumor (nephroblastoma) in the Wilms tumor (nephroblastoma) in the Wilms tumor (n
13、ephroblastoma) in the adult patient : clinical and radiologic manifestationsadult patient : clinical and radiologic manifestationsadult patient : clinical and radiologic manifestationsadult patient : clinical and radiologic manifestations J.AJR ,1989 ,152 (2) :299 - J.AJR ,1989 ,152 (2) :299 - 30213
14、021病 理典型的Wilms瘤由不同比例的原生质、上皮和基质成分组成。NWTS(National Wilms Tumor Study)把病理标本与临床结果联系起来,将多种组织特征分为较好的和不好的预后两组:1.较好的预后肿瘤组织包括未发生间变的所有的Wilms瘤(FH, favorable histology)2.不好的预后(UH, unfavorable histology)一组肿瘤含有局灶性(focal)或弥漫性(diffuse)的间变细胞成分(anaplasia),或含有其他两种非Wilms瘤变种的肿瘤实体肾透明细胞肉瘤(CCSK, clear cell sarcoma of the k
15、idney)和杆状肿瘤(RTK, rhabdoid tumor of the kidney)成分。 史密斯泌尿外科学史密斯泌尿外科学 第第1616版版 肾母细胞瘤肾母细胞瘤肾母细胞瘤肾母细胞瘤 354-357354-357治 疗Treatment Protocol for National Wilms Tumor Study-5The usual approach in most patients is nephrectomy followed by chemotherapy with or without postoperative radiotherapy. CampbellCampbel
16、ls Urology Eighth Edition; Chapter 70 s Urology Eighth Edition; Chapter 70 Pediatric Urologic Oncology; Pediatric Urologic Oncology; WilmsWilmsWilmsWilms TumorTumorTumorTumor 2481-2493 2481-2493相关文献(1) Adult Wilms Tumor: A Monoinstitutional Experience and a Review of the Literature CANCER July 15, 2
17、004 / Volume 101 / Number 2CANCER July 15, 2004 / Volume 101 / Number 2Monica Terenziani, M.D. Filippo Spreafico, M.D. et alUnita di Oncologia Pediatrica, Istituto Nazionaleper lo Studio e la Cura dei Tumori, Milan, Italy. et al相关文献(1)患者:1983-2001年共17人(16-29岁,平均诊断年龄17.5岁),平均随访131月。治疗:依据意大利相关年份治疗WT的指
18、南,同儿童的治疗方案相同。*患者分期依照 NWTS-4 staging system相关文献(1)相关文献(1)17人中有人肿瘤复发或是出现疾病进展,人死亡。复发的患者手术切除后,2人分别随访24个月和152个月无复发,1人随访30月后出现再次复发。平均随访131月中,5年于10年的生存率(OS)比是62.4%,无病生存(DFS)比是45.8%。排除一个弥漫性间变(diffuse anaplasia)患者,5年和10年的OS和DFS比分别是68.7%和50%。结论:作者相信适当的应用儿童Wilms瘤诊断及治疗指南,可以提高成人患者的预后相关文献(2)1.1982年,NWTS第一次报道对31位成
19、人WT患者的治疗结果(1968-1979年)。2.28人行手术治疗,30人行化疗,24人(77.4%)接受术后放疗,51.7%患者处在期和期3.3年存活率是24%,当时儿童患者为74%结论:作者认为成人WT预后差,无论处在哪个期都应该积极的行3联化疗加对肿瘤床放疗(4500 centigrays cGy)。 Byrd RL, Evans AE, DByrd RL, Evans AE, DAngio GJ. Angio GJ. Adult WilmsAdult WilmsAdult WilmsAdult Wilms tumor: effect of combined therapy on tum
20、or: effect of combined therapy on tumor: effect of combined therapy on tumor: effect of combined therapy on survival.survival.survival.survival. J Urol. 1982;127:648 J Urol. 1982;127:648651651. .相关文献(3)1.1990年,NWTS第一次报道通过采用儿童治疗方案获得较好的预后。2.1979-1987年,选择27位成人WT患者。3.4人为期合并间变肿瘤, 期和期患者超过50%。25个患者行化疗,20个患
21、者(74%)行放疗。4.3年的存活率为67%,除去间变肿瘤患者3年存活率为79%。结论:I期行2联化疗,无需放疗;对II、III、IV期患者应行3联化疗加肿瘤床放疗(2000 cGy)。Arrigo S, Beckwith JB, Sharples K, et al. Arrigo S, Beckwith JB, Sharples K, et al. Better survival after combined modality care for Better survival after combined modality care for Better survival after com
22、bined modality care for Better survival after combined modality care for adults with Wilmsadults with Wilmsadults with Wilmsadults with Wilms tumor. A report from the National Wilms tumor. A report from the National Wilms tumor. A report from the National Wilms tumor. A report from the National Wilm
23、s Tumor Study. Tumor Study. Tumor Study. Tumor Study. Cancer. 1990; Cancer. 1990; 66:82766:827830.830.相关文献(4)1.1994年,法国报道22位大于16岁的患者,治疗时间超过19年。2.所有患者行肾切除;15位患者接受放化疗;6个患者接受化疗;1位患者仅接受了放疗。3.平均随访100个月,其中55%患者仍存活,其中45%的患者无瘤生存结论:超过II期(包括II期)患者建议行3联化疗加放疗。Kattan J, Tournade MF, Culine S, et al. Kattan J, To
24、urnade MF, Culine S, et al. Adult WilmsAdult WilmsAdult WilmsAdult Wilms tumour: review of 22 cases. tumour: review of 22 cases. tumour: review of 22 cases. tumour: review of 22 cases. Eur J Eur J Cancer. 1994;30:1778Cancer. 1994;30:17781782.1782.相关文献(5)1.2003年,德国在国际儿童肿瘤学会(SIOP)上报道30例成人WT的治疗结果。治疗方案根
25、据the SIOP 93-01 study.2.6位患者(13%)诊断为高度危险肿瘤;10人(33%)被发现远处转移。3.所有患者行手术和化疗,其中14人同时接受放疗4.平均随访4年,无瘤生存和生存率(OS)分别为57%和83%。结论:作者认为通过儿童WT治疗方案对成人患者治疗可以获得好的预后。 Reinhard H, Aliani S, Leuschner I, et al. Reinhard H, Aliani S, Leuschner I, et al. WilmsWilmsWilmsWilms tumor in adults. Results of the SIOP 93-01 stu
26、dy tumor in adults. Results of the SIOP 93-01 study tumor in adults. Results of the SIOP 93-01 study tumor in adults. Results of the SIOP 93-01 study abstract.abstract.abstract.abstract. Med Pediatr Oncol. 2003;38:266. Med Pediatr Oncol. 2003;38:266.谢 谢!Denys-Drash syndromeOne specific association o
27、f male pseudohermaphroditism, renal mesangial sclerosis, and nephroblastoma is known as the Denys-Drash syndrome (DDS)Drash A, Sherman F, Hartmann WH, Blizzard RM: Drash A, Sherman F, Hartmann WH, Blizzard RM: A syndrome of pseudohermaphroditism, A syndrome of pseudohermaphroditism, A syndrome of ps
28、eudohermaphroditism, A syndrome of pseudohermaphroditism, Wilms tumor, hypertension and degenerative renal disease.Wilms tumor, hypertension and degenerative renal disease.Wilms tumor, hypertension and degenerative renal disease.Wilms tumor, hypertension and degenerative renal disease. J Pediatr 197
29、0;76:585 J Pediatr 1970;76:585593.593.Genitourinary anomaliesGenitourinary anomalies (renal fusion anomalies, cryptorchidism, hypospadias) are present in 4.5% of patients with Wilms tumor. Breslow N, Olshan A, Beckwith JB, Green DM: Breslow N, Olshan A, Beckwith JB, Green DM: Epidemiology of WilmsEp
30、idemiology of WilmsEpidemiology of WilmsEpidemiology of Wilms tumor. tumor. tumor. tumor. Med Pediatr Med Pediatr Oncol 1993;21:172Oncol 1993;21:172181.181.WAGR syndromeWAGR syndrome is characterized by Wilms tumor, aniridia, genital anomalies, mental retardation. Clericuzio CL: Clericuzio CL: Clini
31、cal phenotypes and Wilms tumor.Clinical phenotypes and Wilms tumor.Clinical phenotypes and Wilms tumor.Clinical phenotypes and Wilms tumor. Med Pediatr Oncol 1993;21:182 Med Pediatr Oncol 1993;21:182187.187.Beckwith-Wiedemann syndrome (BWS)BWS is characterized by excess growth at the cellular, organ
32、 (macroglossia, nephromegaly, hepatomegaly), or body segment (hemihypertrophy) levels.*Beckwith JB: Beckwith JB: Macroglossia, omphalocele, adrenal cytomegaly, gigantism and hyperplastic Macroglossia, omphalocele, adrenal cytomegaly, gigantism and hyperplastic Macroglossia, omphalocele, adrenal cyto
33、megaly, gigantism and hyperplastic Macroglossia, omphalocele, adrenal cytomegaly, gigantism and hyperplastic visceromegaly.visceromegaly.visceromegaly.visceromegaly. Birth Defects 1969;5:188 Birth Defects 1969;5:188196.196.Wiedemann H: Wiedemann H: Tumors and hemihypertrophy associated with the Wied
34、emann-Beckwith syndrome.Tumors and hemihypertrophy associated with the Wiedemann-Beckwith syndrome.Tumors and hemihypertrophy associated with the Wiedemann-Beckwith syndrome.Tumors and hemihypertrophy associated with the Wiedemann-Beckwith syndrome. Eur J Pediatr 1983;141:129Eur J Pediatr 1983;141:129. .意大利治疗方案*人有了知识,就会具备各种分析能力,明辨是非的能力。所以我们要勤恳读书,广泛阅读,古人说“书中自有黄金屋。”通过阅读科技书籍,我们能丰富知识,培养逻辑思维能力;通过阅读文学作品,我们能提高文学鉴赏水平,培养文学情趣;通过阅读报刊,我们能增长见识,扩大自己的知识面。有许多书籍还能培养我们的道德情操,给我们巨大的精神力量,鼓舞我们前进。