Patologia cilor biliare intra/extra hepatice i a veziculei biliareDelia Ciobanu

Glande LuschkaSinusuri Rokitansky-Aschoff

Anomaliile congenitaleColesteroloza veziculei biliareColelitiazaColecistiteleTumorile cilor/veziculei biliare

Anomalii congenitaleVezicula biliar:Anomalii de form i dimensiune: Vezicula frigian - angularea fundusului veziculei biliareAgenezia/hipoplazia veziculei biliareDuplicarea/triplicarea veziculei biliareVezicula multiseptat 3-10 septuri tapetate de epiteliu cilindric (n sticl de ceas/transversal)Diverticulii/pseudodiverticuli ai veziculei biliareAnomalii de localizare:Vezicula inclus n parenchimul hepaticVezicula plonjantHeterotopia cu mucoas gastric, pancreatic, hepatic, tiroidian, SR

Anomalii congenitale

Vezicula phrygian

Heterotopia de mucoas gastric

Anomalii congenitaleCi biliare:Atrezia cilor biliare intra i extrahepaticeChisturile de canale biliare:Chist de canal coledocColedococelulMaladia Caroli

Atrezia veziculei biliare i a cilor biliareAtrezia cilor biliare intra/extra hepatice Manifestat precoce la copil prin icter colestatic persistent Evoluie ctre ciroz biliar secundar

Atrezia veziculei biliare i a cilor biliare Incidena: 1/20 000 and 1/3 100 nou nscui, Asia i arile din jurul oceanului Pacific sunt cele mai afectate. 1/18 000 nou nscui n Europa. Sexul feminin este mai afectat dect cel masculin.

Chisturile de ci biliare

Maladia Caroli Multiple chisturi aprute n cile biliare intrahepatice care nu comunic cu lumenul cilor biliareficat cu aspect polichistic chisturi multiple canalul coledoc Complicaii colengit/abcese colangitice

Colesteroloza veziculei biliare25% din cazurile de colecistectomieAcumularea colesterolului esterificat n macrofagele din lamina propria (celule xantice)Macroscopic: vezicula fragMicroscopic:Celule dispuse sub forma unor grmezi, subepitelial, cu citoplasm spumoas, nucleu centralPolipi colesteroloziciPrezint birefringen n lumina polarizatInflamaia redus/absentEtiologie:Suprasaturarea bilei n colesterolAlterri ale transportului normal al colesterolului

Colesteroloza veziculei biliare

Colesteroloza veziculei biliare

Colesteroloza veziculei biliare

ColelitiazaCea mai frecvent patologie biliarAsociat/nu cu hepatolitiaz/coledocolitiazCalculi:Colesterol:75-80%Puri (90%)/micti (60-70% colesterol)UniciRotund/ovalari, culoare galben, suprafa netedAspect radiar pe seciuneFactori favorizani: genetici/sarcini repetate/boli ileale/obezitateaMecanismele formrii:Dereglare a raportului colesterol/acizi biliari i lecitine; col+glicoprotideMicti multipli, faetai, centru pigmentat de bilirubinat de Ca + pturi concentrice de col

ColelitiazaCalculi:Pigmentari:10-25%Colesterol sub 25%Bilirubinat de Ca negri/brunintotdeauna multipliiNegri suprafa lucioas, radiari pe seciune, relativ duri, se formeaz n bila sterilBruni - staz/infecii biliare; suprafa solzoas, care se detaeaz n lambouri, duritate micMacanism: glucuronidazele bacteriene care degradeaz bilirunina neconjugatCarbonat de calciu (calculi/nisip biliar)Alb-glbui galben-verzui, radioopaci

Colelitiaz

Colelitiaza

Colelitiaza - complicaiiCalculii exercit aciune iritativ asupra mucoasei:InflamaiePerforaie fistulecolecisto-duodenalecolecisto-colicecoledoco-duodenaleHidrops vezicularobliterarea coletului vezical/cisticvezicula destins, perete gros/fibros, lumenul conine un lichid transparent/mucoid

ColecistiteleInflamaii ale colecistului acute/croniceColecistitele acute:Etiologie:90% calculoaseNe-calculoase: infecii ascendente ci biliare Giardia/lamblia, malformaii ci biliareMacroscopic:Colecistita acut de intensitate moderat colecist destins, mucoasa de culoare roie cu arii hemoragice, seroasa acoperit cu depozite de fibrin, peretele ngroat peste 2 cm edem i inflamaie; coninut purulent+bil Colecistita flegmonoas calculoas (ischemie prin comprimarea vascularizaiei/flor bacterian asociat)Colecistita gangrenoas perete negru-verzui, friabil, perforaii/peritonitMicroscopic:Colecistita de intensitate redus: mucoas cu exudat purulent, arii de ulceraie ale mucoaseiColecistita acut flegmonoas inflamaie acut n toate pturileColecistita gangrenoas - + tromboze vasculareComplicaii: abces pericolecistic/peritonita

Colecistita acut

Colecistita acut

Colecistita acut

Colecistita cronic95% asociat cu colelitiazMacroscopic:Form hipertroficForm atroficMicroscopic:Inflamaie cronic cu celule mononucleate n peretele colecistuluiFibroz parietalSinusuri Rokitanski-Aschoff (90%)Metaplazie: piloric, gastric, intestinalFoliculi limfoizi colecistit cronic folicular

Colecistit cronicHipertrofia peretelui colecistuluiMucocel al veziculei biliare - peretele subire, suprafaa neted i secreia mucoas incolor (hidrops vezicular)

Colecistita cronic

Vezicula de porelan

Tumorile veziculei biliareTumori benigne:Polipul mucos:AdenomatoiHiperplaziciColesteroloziciInflamatorFibrosAdenomiozaHemangiom/leiomiom/lipom/paragangliom/limfangiomTumori maligne:Carcinomul de vezicul biliarSarcoameleCarcinosarcoamele (tumori mixte)CarcinoidulTumori secundare

Polip adenomatos tubular/papilar

Adenomioza

Carcinomul veziculei biliareLocul 5 n cadrul tumorilor gastro-intestinaleFrecven:Decada 6-7F/B-3/1Antecedente: colelitiaz (75%)/colecistit cronic (50%)Macroscopic:Localizare de elecie n zona fundic, tumoare vegetant/infiltrativMicroscopic:Adenocarcinom (75-85%)Metaplazie scuamoas (5-10%)/carcinom scuamos (20-50%)Carcinom papilar (4-20%)Coloid (4-7%)Adenocarcinom pleomorf cu celule gigante (13%)Carcinom cu celule n inel cu pecete (3%)Evoluie:Supravieuire la 5 ani 4-12%Forma papilar pronostic bunDiseminare limfatic (ganglioni regionali)/hematogen (hepatice)

Carcinomul de vezicul biliar

AdenocarcinomBine difereniatModerat difereniat

Carcinom scuamocelular

Clasificarea OMS tumori vezicalePrimary Tumor (T)TX Primary tumor cannot be assessedT0 No evidence of primary tumorTis Carcinoma in situT1 Tumor invades lamina propria or muscular layer T1a Tumor invades lamina propria T1b Tumor invades muscular layer T2 Tumor invades perimuscular connective tissue; no extension beyond serosa or into liverT3 Tumor perforates the serosa (visceral peritoneum) and/or directly invades the liver and/or one other adjacent organ or structure, such as the stomach, duodenum, colon, pancreas, omentum, or extrahepatic bile ducts T4 Tumor invades main portal vein or hepatic artery or invades two or more extrahepatic organs or structuresRegional Lymph Nodes (N)NX Regional lymph nodes cannot be assessedN0 No regional lymph node metastasisN1 Metastases to nodes along the cystic duct, common bile duct, hepatic artery, and/or portal veinN2 Metastases to periaortic, pericaval, superior mesenteric artery, and/or celiac artery lymph nodes

PROGNOSTIC FEATURESIn as many as 50% of cases, gallbladder cancers are discovered at pathologic analysis after simple cholecystectomy for presumed gallstone disease. Five-year survival is 50% for patients with T1 tumors. Patients with T2 tumors have a 5-year survival rate of 29%, which appears to be improved with more radical resection. Patients with lymph node metastases (Stage IIIB or higher) or locally advanced tumors (Stage IVA or higher) rarely experience long-term survival. The site-specific prognostic factors include histologic type, histologic grade, and vascular invasion. Papillary carcinomas have the most favorable prognosis. Unfavorable histologic types include small cell carcinomas and undifferentiated carcinomas. Lymphatic and/or blood vessel invasion indicate a less favorable outcome.

PROGNOSTIC FEATURESHistologic grade also correlates with outcome.Patients with T2T3 cancers discovered at pathologic analysis are usually offered a second operation for radical resection of residual tumor. This may include nonanatomic resection of the gallbladder bed (segments IVB and V of the liver) or more formal anatomic resection such as a right hepatectomy. Resection of the biliary tree is dependent on surgical decision making at the time of the definitive procedure and may be based on cystic duct margin status. Staging classification should be reported for tumors removed by either a single operation or a staged surgical procedure (cholecystectomy followed by definitive resection).In cases where the surgical procedure was staged, it should be noted whether the cholecystectomy was performed laparoscopically or via an open approach.

Factori prognosticiPROGNOSTIC FACTORS (SITE-SPECIFIC FACTORS)(Recommended for Collection)Required for staging NoneClinically significant Tumor locationSpecimen typeExtent of liver resectionFree peritoneal side vs. hepatic side for T2

Carcinoamele cilor biliareInciden:0,012-0,54% autopsii/ 0,3-1,8% operaii tract biliarDecada 6-7Antecedente:Chirurgie biliar primar (20%)Infecii: Clonorchis sinensisFibroza chistic familialColelitiaza (33%)Macroscopic:Tumori Klatskin (tumori hilare) fuziunea canalelor hepatice40-55% proximale20-25% medii20-25% distaleVegetante/infiltrativeMicroscopic:Adenocarcinoame 90-95%Adenoscuamos 6,5%ScuamocelularEvoluie: rapid, metastaze limfatice/nu hematogene pacienii se pierd rapid

Carcinom ci biliare

Carcinom ci biliare

Clasificarea OMS tumori ci biliarePrimary Tumor (T)TX Primary tumor cannot be assessedT0 No evidence of primary tumorTis Carcinoma in situT1 Tumor con fi ned to the bile duct, with extension up to the muscle layer or fi brous tissueT2a Tumor invades beyond the wall of the bile duct to surrounding adipose tissue T2b Tumor invades adjacent hepatic parenchyma T3 Tumor invades unilateral branches of the portal vein or hepatic artery T4 Tumor invades main portal vein or its branches bilaterally; or the common hepatic artery; or the second-order biliary radicals bilaterally; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvementRegional Lymph Nodes (N)NX Regional lymph nodes cannot be assessedN0 No regional lymph node metastasisN1 Regional lymph node metastasis (including nodes along the cystic duct, common bile duct, hepatic artery, and portal vein) N2 Metastasis to periaortic, pericaval, superior mesenteric artery, and/or celiac artery lymph nodes

PROGNOSTIC FEATURESPatients who undergo surgical resection for localized perihilar cholangiocarcinoma have a median survival of approximately 3 years and a 5-year survival rate of 20% to 40%. In carefully selected patients with primary sclerosing cholangitis and early-stage perihilar cholangiocarcinoma, preliminary data report excellent results with neoadjuvant chemoradiation and liver transplantation.Complete resection with negative histologic margins is the major predictor of outcome, and liver resection is essential to achieve negative margins. Factors adversely associated with survival include high tumor grade, vascular invasion, lobar atrophy, and lymph node metastasis. Papillary morphology carries a more favorable prognosis than nodular or sclerosing tumors.PROGNOSTIC FACTORS (SITE-SPECIFIC FACTORS)(Recommended for Collection)Required for staging NoneClinically significant Tumor locationPapillary variantTumor growth patternPrimary sclerosing cholangitisCA 19-9