阑尾上皮肿瘤的病理诊断课件

1、阑尾上皮肿瘤病理Epithelial Tumor 上皮肿瘤Premalignant lesions 癌前病变Adenoma （腺瘤）: tubular , tubulovillous and villousDysplasia （不典型增生） : Intraepithelial neoplasia （上皮内瘤）, LG and HGSerrated lesions （锯齿状病变）: HP, SSA/P and TSACarcinomaAdenocarcinomaMucinous adenocarcinoma 粘液腺癌Low grade appendiceal mucinous neoplasm

2、低级别阑尾粘液肿瘤Signet ring cell carcinoma 印戒细胞癌Non-mucinous adenocarcinoma 非粘液腺癌Undifferentiated carcinomaNeuroendocrine Neoplasm 神经内分泌肿瘤NET (G1, G2)NEC: large and small cellMixedGoblet cell 杯状细胞Tubular carcinoid 管状EC cell, L cell etcWHO classification of appendiceal tumor （阑尾肿瘤分类）Hyperplastic lesions （增生

3、性病变）Polyp （息肉）: uncommondiffuse hyperplasia （弥散性增生） Sessile serrated adenoma/polyp （无蒂锯齿状）Same histological criteriaSerrated lesions （锯齿状病变）Hyperplastic polypDiffuse hyperplastic changesSessile Serrated AdenomaTends to be villous growth pattern （绒毛）No mucin dissection, limited above mucosa musculari

4、s （没有切割， 在粘膜肌层上）Commonly cause lumen dilatation: cystadenoma; clinically mucoceleAdenoma/cystadenoma （囊腺瘤）Tubular Adenoma/LG dysplasiaTubular Adenoma with HG dysplasiaTubular Adenoma with HG dysplasiaDefinition: invasion beyond muscularis mucosaIncidence: 0.1-0.2% of appendectomy; 0.2 per 100,000Eld

5、er patientsEtiology: unknownmay associated with UC and tumor in large intestineAdenocarcinoma of appendixNon-mucinous: Similar to colorectal adenocarcinomaMucinous adenocarcinomaaccounts for 50%Low grade and high gradeCK20, CDX2, and variable CK75 yrs survival 40-50%Signet ring cell carcinomaClassif

6、ication of AdenocarcinomaNon-mucinous ADC阑尾粘液癌分类GradeTMN classificationArchitectureCellular featuresMucinMitosisLow grade (LAMN)Mucinous, LGresembling adenoma or SSA; “broad front invasion”: no desmoplasticLow grade dysplasia or bland cytologyLarge mucin vacuoleRareMucinous ADCMucinous, HGSimilar to

7、 colorectal ADC, desmoplastic HG dysplasiaVariable; may see signet ring cellcommonWHO classifications of tumor: of digestive system: p121Mucinous ADC, HG占粘液肿瘤大多数是腺癌Could cause pseudomyxoma peritonei （腹膜假粘液瘤）(could be acellular mucin dissection)Have well differentiated epithelial lining, minimum cyto

8、logical atypiaLow grade appendiceal mucinous neoplasm (LAMN)LAMNLAMNLAMNClinical term（临床诊断）: mucinous ascites or mucinous deposits in the peritoneal cavity （粘液腹水或粘液结节在腹腔内）Causes:Most due to appendiceal neoplasmOther GI and GYN tract neoplasmsBenign rupture: perforation of diverticula and appendixPsu

9、edomyxoma peritonei （腹膜假粘液瘤）Case Number appendicealOvarian tumor presentconclusionYoung et alN=222222Appendiceal primary, ovary secondarySeidman et aln=2522 of 2511/25Independent origin of ovary and appendixPrayson et asF/M 9/1019/194 of 994% from appendixCautrecause et alN=5N=5583% have identitical

10、 k-ras mutationK-Ras mutationChauqua et al121212Appendix primary75% LOH chromosome 5 and 17腹膜假粘液瘤哪里来?Intact laminar propria和阑尾憩室鉴别Muscle or Schwann cell proliferationClassification of psuedomyxoma peritoneiGradeTMN classificationArchitectureCellular featuresMucinMitosisLow gradeMucinous, LGCells for

11、m strips or small island; scantly and/or acellular mucinSingle layers, papillary tufting; nuclei small and regularVariableRareHigh gradeMucinous, HGStrips, small islands or cribriform. High cellularity; invasion into other organsHG dysplasiaVariable; may see signet ring cellcommonWHO classifications

12、 of tumor: of digestive system: p123如果肿瘤细胞没有穿透粘膜肌层： 腺瘤; 预后好， 完全切除后治愈 (submit the appendix entirely)肿瘤细胞过粘膜肌层： 腺癌LG and HG肿瘤细胞在粘膜， 但又粘液 (acellular or scant cellularity, bland cytology) deposit 在粘膜外LGMN (有细胞）mucin dissection limited in the appendiceal wall: cured by excisionAcellular mucin: controvers

13、ial, differentiate from rupture; clear cut adenoma, LGMNTMN staging: T4a, serosal 右下腹; M1a, PMP beyond right quadrantPrognosis associated with the histological grade and/or invasion deep to the peritoneal surfaceDebulking of peritoneal mucus prolong survival, particular in LGMNNotes for WHO classifi

14、cationThree-tiered system (mucinous adenocarcinoma): Low grade, grade G1, well differentiatedHigh grade， grade G2 (moderately) or grade G3 (poorly)No defined histological criteriaT3: non-continuous tumor nodule or tumor depositT4a and T4bPeritoneal involvement limited to right quadrant: T4M1a (PMP)

15、and M1b: N, M,GStage IVa: Any T, N0, M1a, G1Stage IVb: Any T, N0, M1a, G2,G3 Any T, N1/2, M1a, any GStage IVc: Any T, Any N, M1b, any GOverman 2014: stage IV, moderately and poorly differentiated mucinous adenocarcinomas have distinctly different prognosisAJCC classification (7th edition)经常没有原位肿瘤, 可

16、以发生于粘膜下层或深部粘膜: 大小比浸润深度意义大 T stage:T1: 2 cmT2: 2 cm to 4cm or extension to cecumT3: more than 4cm or to the ileumT4: directly invades adjacent organsHistological grade: as other neuroendocrine tumor: G1, G2, G3Goblet cell carcinoid uses adenocarcinoma criteriaSerum chromogranin: A prognostic markerNeuroendocrine tumor内分泌肿瘤Goblet cell carcinoid 杯状细胞类癌:主要在粘膜下层一般不侵犯粘膜不形成明显肿块Small round, signet ring like cellsNeuroendocrine markers positive; CEA, CK19, CK20 and MUC2Mixed carcinomaSignet ring or