肾细胞癌的分类-compressed.ppt

,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,Classification of Renal Carcinoma,Renal Unit,CK19,THP,CD10,、,RCC,UEA-1,Renal Carcinoma,Clear Cell Carcinoma,Granular cell carcinoma,Clear Cell Carcinoma,Chromophobe,Carcinoma,Papillary Carcinoma,Type I,Type II,Collecting duct carcinoma,Oncocytoma,Metanephric Adenoma,、,Adenofibroma,Unclassified and unusual carcinoma,Case 1,37 yr male.History of cerebellar hemangioblastoma,renal tumor and cyst,Gross,：,Tumor diameter 5X5X2.5cm,，,cut surface yellow,Some cysts in the tumor,Risk factor for Von Hippel Lindau syndrome,Younger than 50 years,Multifocal unilateral or bilateral tumors,Family history of renal cancer or other VHL related tumor,Germline mutation analysis,Missense mutation of VHL gene on 3p25-26,Diagnostic Criteria for VHL Syndrome,No family history,Hemangioblastoma of CNS or retina,+,VHL associated extraneural tumors,With family history,Hemangioblastoma of CNS,or,retina,or,VHL associated extraneural tumors,Case 1,Diagnosis,VHL associated clear cell renal cell carcinoma,VHL subtype,HB,RCC,PCC,Mutation,Type 1,+,+,-,deletion/truncation,Type 2A,+,-,+,Tyr98His/Tyr112His,Type 2B,+,+,+,Missense,Type 2C,-,-,+,Leu188Val,Ser80Leu,HB,：,Hemangioblastoma,；,RCC,：,Renal cell carcinoma,；,PCC,：,Pheochromocytoma,Pathogenesis,Cohen H and McGovern FJ,N Engl J Med,2005;353:23,pVHL in tumor initiation,Sporadic Clear Cell Carcinoma,Pathogenesis,Cohen H and McGovern FJ,N Engl J Med,2005;353:23,Fuhrman Nuclear Grade,（,4 tiered,）,Note,：,Apply in clear cell carcinoma and papillary carcinoma,Grade,Diameter of Nuclei,Nuclei,Nucleoli,Other Features,1,10m,Round,Absence or inconspicuous,2,15m,Finely granular open chromatin,inconspicuous,400,3,20m,irregular,large,100,4,20+m,Pleomorphism,large,Clump chromatin,spindle cell,Multilocular Cystic Renal Cell Carcinoma,Multilocular Cystic Renal Cell Carcinoma,Modern Pathol 2010 23,931936,Most of the cases showed chromosome 3p deletion.,Very good survival,Hereditary RCC,Hereditary leiomyomatosis and RCC,Birt-Hogg-Dub syndrome,Hyperparathyroidism-Jaw tumor syndrome,Familial Papillary Thyroid Cancer,Constitutional chromosome 3 translocations,Hereditary papillary renal cancer,Hereditary leiomyomatosis and RCC,Leomyoma or leomyosarcoma of skin or uterus,Unilateral papillary renal carcinoma,Fumarate Hydratase,（,FH,）,mutation 1q42,Birt-Hogg-Dub Syndrome,Facial fibrofolliculoma and tricodiscoma,Lung cyst with risk of spontaneous pneumothorax,Colonic polyps,Renal tumors:oncocytoma or chromophobe carcinoma,Folliculin,（,BHD,）,gene mutation 17p11.2,Birt-Hogg-Dub Syndrome,Hyperparathyroidism-Jaw Tumor Syndrome,Autosomal dominant,Parathyroid adenomas(carcinoma),、,Ossifying fibroma of the maxilla or mandible,、,Renal cysts or renal tumor,Wilms tumor,、,mixed epithelial-stromal tumor,、,papillary carcinoma,HPT-JT,（,parafibromin,）,mutation 1q21q32,J Intern Med,2003 253(6):634,Familial Papillary Thyroid Cancer,Familial PTC Associated with Renal Cell Neoplasia,1q21,Not c-Met associated Hereditary renal papillary cacinoma,Not Cowden syndrome,Not MEN2A,Case 2,45 yr male,Right kidney mass,Papillary Renal Carcinoma,Type 1,Papillae covered by small cells with scanty cytoplasm,Single layer on the papillary basement membrane.,Type 2,Higher nuclear grade with eosinophilic cytoplasm,Pseudostratified nuclei on papillary cores.,Type 1,Type 2,Case 2,Diagnosis,Papillary Renal Cell Carcinoma,Type I,Prognosis of PRCC,Conventional papillary renal carcinoma,75%with duplication of chromosome 7,proto-oncogene,MET,Cohen H and McGovern FJ,N Engl J Med,2005;353:23,Papillary Renal Cancer involved in Hereditary Syndromes,Hereditary papillary renal cancer,Hereditary leiomyomatosis and RCC,Birt-Hogg-Dub syndrome,Hyperparathyroidism-Jaw tumor syndrome,Familial Papillary Thyroid Cancer,Papillary Adenoma,GrignonDJ,EbleJN.Papillary and metanephric adenomas of the kidney.Semin Diagn Pathol.1998;15:4153.,Definition,Tumors with papillary or tubular architecture of low nulear grade and 5 mm in diameter or smaller,Papillary epithelial neoplasm of low malignant potential,Grade 1 papillary tumors between 0.5-2cm are strictly defined as carcinoma,Tumor Diameters and Metastasis at Autopsy(871 patients),Max tumor diameter,n,M1 or N1,1-3 cm,208,7(3.4%),1-2.5 cm,177,4(2.2%),1-2.0 cm,154,2(1.3%),1.0 cm,396,0,Grignon and Eble.Semin Diag Pathol.1998;15:41,Proposed Developmental Steps of PRCC,KL Wang,et al.Hum Pathol 2007 38(3):239,Case 3,33 yr,female,Right kidney mass,2X1.5X0.5cm,surface yellow,Clear-Cell Papillary Renal Cell Carcinoma,Clear-cell tubulopapillary renal cell carcinoma,Renal angiomyoadenomatous tumor,Renal cell carcinoma with prominent leiomyomatous proliferation,Clear cell renal cell carcinoma with diffuse cytokeratin 7 immunoreactivity,Clear-Cell Papillary Renal Cell Carcinoma,Cytokeratin 7+,carbonic anhydrase IX(CA9)+,HIF-1,+,GLUT-1+,CD10-,P504S-,TFE3-,Absence of VHL gene alteration,Case 4,25 years old female,CT revealed a mass in right kidney,Nephrectomy was performed,TFE-3,Renal carcinomas associated with Xp11.2 translocations,Papillary architecture comprised of clear cells,Nested architecture,Cells with voluminous granular eosinophilic cytoplasm,Psammoma bodies,Xp11 Translocation Carcinoma,Heimann et al.Fusion of a novel gene,RCC17,to the TFE3 gene.Cancer Res 2001;61:4130,Argani et al.Primary renal neoplasms with,ASPL-TFE3,gene fusion of alveolar soft part sarcoma.Am J Surg Pathol 2001;159:179,Argani et al.,PRCC-TFE3,renal carcinomas.Am J Surg Pathol 2002;26:1553,Case 3,Diagnosis,Renal carcinomas associated with Xp11.2 translocations,Microphthalmia Transcription Factor/TFE Family,MiTF,TFE3,TFEB,TFEC,Homologous DNA binding domain,Recognize identical DNA sequences,Activate transcription of similar genes in vitro,Am J Surg Pathol 2005,t(6;11)(p21;q12)Renal Carcinoma,Ages 6-53 years(median 18 yr),Morphology,Epithelioid cells,predominantly clear cytoplasm,IHC,Minimal cytokeratins expression,All HMB45,Melan-A(+),Aberrant positive of TFEB transcription factor,Diagnostic Use of MiTF/TFE Family Protein,Genes,IHC Assays,Neoplasms,MiTF,Yes,Malignant melanoma,TFE3,Yes,Alveolar soft part sarcoma,Xp11 translocation carcinoma,TFEB,Yes,t(6;11)(p21;q12)Renal Carcinoma,TFEC,No,?,Chromophobe Renal Cell Carcinoma,Large pale cells with prominent cell membranes,Perinuclear halos,Eosinophilic variant,Mucinous tubular and spindle cell carcinoma,Tightly packed,small,elongated tubules separated by pale mucinous stroma,The parallel tubular arrays often have a spindle cell configuration,Cuboidal or oval nulear shapes and low-grade features,Positive for EMA,AE1/AE3,CK7,CK19,34E12 and AMACR,MTSCC-Loopoma?,Collecting Duct Carcinoma,Collecting Duct Carcinoma,Arises in the principal cells of the collecting ducts of Bellini,Complex tubulopapillary architecture,Pronounced stromal desmoplasia,Complex interanastomosing structures,CK19,、,34bE12,、,UEA-1+,CD10,、,Villin-,CK19,Low Grade Collecting Duct Carcinoma-,Tubulocystic carcinoma,MacLennanGT,et al.,Low-grade collecting duct carcinoma,of the kidney:report of 13 cases of low-grade mucinous tubulocystic renal carcinoma of possible collecting duct origin.,Urology,1997;50:679-684.,AminMB,et al.,Tubulocystic carcinoma,of the kidney:Clinicopathologic analysis of 29 cases of a distinctive rare subtype of renal cell carcinoma.,Mod Pathol,2004;17:137A,Tubulocystic carcinoma,Tubules and cystic structures of markedly variable size,separated by delicate septa.“spider web-like”,Tubules are lined by a single layer of low cuboidal epithelial cells with hobnail appearance,IHC similar to collecting duct carcinoma,Renal Medullary Carcinoma,Strongly associated with sickle cell hemoglobinopathies,Typically located in the renal medulla,May arise in the terminal collecting ducts and the adjacent papillary epithelium,Reticular or microcystic growth pattern,Tubule formation and growth in diffuse sheets or solid nodules,Pleomorphic nuclei,prominent nucleoli,and eosinophilic cytoplasm,IHC positive for CEA,EMA,CK19,topoisomerase IIa,Renal Medullary Carcinoma,Acquired Cystic Disease-Associated Renal Cell Carcinoma,End-stage renal disease(ESRD)50%,Acquired cystic kidney disease(ACKD)3-7%,Renal cell carcinoma,Acquired Cystic Disease-Associated Renal Cell Carcinoma,40%are classic clear cell,papillary or chromophobe RCC,Acquired cystic disease-associated RCC,Clear cell papillary RCC of end-stage kidneys,Acquired Cystic Disease-Associated Renal Cell Carcinoma,Often multifocal and bilateral,Solid sheet-like,papillary,acinar,cribriform and tubulocystic patterns,Abundant intratumoral calcium oxalate crystals within luminal structures and in the stroma,Purely Sarcomatoid RCC,CAM 5.2,Can be presented in different histological RCC,Worse survival than conventional RCC,Other Rare Epithelial Neoplasms,Spiradenocylindroma,Adenoid cystic carcinoma,Thyroid follicular carcinoma-like carcinoma,Thanks For Your Attention,资料可以编辑修改使用,学习愉快！,课件仅供参考哦，,实际情况要实际分析哈！,感谢您的观看,