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Patologia cilor

biliare intra/extra
hepatice i a veziculei
biliare
Delia Ciobanu

Glande Luschka

Sinusuri Rokitansky-Aschoff

Anomaliile congenitale
Colesteroloza veziculei biliare
Colelitiaza
Colecistitele
Tumorile cilor/veziculei biliare

Anomalii congenitale
Vezicula biliar:
Anomalii de form i dimensiune:
Vezicula frigian - angularea fundusului veziculei biliare
Agenezia/hipoplazia veziculei biliare
Duplicarea/triplicarea veziculei biliare
Vezicula multiseptat 3-10 septuri tapetate de epiteliu
cilindric (n sticl de ceas/transversal)
Diverticulii/pseudodiverticuli ai veziculei biliare
Anomalii de localizare:
Vezicula inclus n parenchimul hepatic
Vezicula plonjant
Heterotopia cu mucoas gastric, pancreatic, hepatic,
tiroidian, SR

Anomalii congenitale

Vezicula phrygian

Heterotopia de mucoas
gastric

Anomalii congenitale
Ci biliare:
Atrezia cilor biliare intra i extrahepatice
Chisturile de canale biliare:
Chist de canal coledoc
Coledococelul
Maladia Caroli

Atrezia veziculei biliare i a


cilor biliare

Atrezia 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 biliare
ficat cu aspect polichistic chisturi multiple canalul coledoc
Complicaii colengit/abcese colangitice

Colesteroloza veziculei
biliare
25% din cazurile de colecistectomie
Acumularea colesterolului esterificat n
macrofagele din lamina propria (celule xantice)
Macroscopic: vezicula frag
Microscopic:

Celule dispuse sub forma unor grmezi, subepitelial, cu


citoplasm spumoas, nucleu central
Polipi colesterolozici
Prezint birefringen n lumina polarizat
Inflamaia redus/absent

Etiologie:

Suprasaturarea bilei n colesterol


Alterri ale transportului normal al colesterolului

Colesteroloza veziculei biliare

Colesteroloza veziculei
biliare

Colesteroloza veziculei
biliare

Colelitiaza
Cea mai frecvent patologie biliar
Asociat/nu cu hepatolitiaz/coledocolitiaz
Calculi:
Colesterol:

75-80%
Puri (90%)/micti (60-70% colesterol)
Unici
Rotund/ovalari, culoare galben, suprafa neted
Aspect radiar pe seciune
Factori favorizani: genetici/sarcini repetate/boli
ileale/obezitatea
Mecanismele formrii:

Dereglare a raportului colesterol/acizi biliari i lecitine;


col+glicoprotide
Micti multipli, faetai, centru pigmentat de bilirubinat de Ca +
pturi concentrice de col

Colelitiaza
Calculi:

Pigmentari:

10-25%
Colesterol sub 25%
Bilirubinat de Ca negri/bruni
ntotdeauna multiplii
Negri suprafa lucioas, radiari pe seciune,
relativ duri, se formeaz n bila steril
Bruni - staz/infecii biliare; suprafa solzoas, care
se detaeaz n lambouri, duritate mic
Macanism: glucuronidazele bacteriene care
degradeaz bilirunina neconjugat

Carbonat de calciu (calculi/nisip biliar)


Alb-glbui galben-verzui, radioopaci

Colelitiaz

Colelitiaza

Colelitiaza - complicaii
Calculii exercit aciune iritativ asupra
mucoasei:
Inflamaie
Perforaie fistule
colecisto-duodenale
colecisto-colice
coledoco-duodenale

Hidrops vezicular
obliterarea coletului vezical/cistic
vezicula destins, perete gros/fibros, lumenul conine
un lichid transparent/mucoid

Colecistitele
Inflamaii ale colecistului acute/cronice
Colecistitele acute:
Etiologie:

90% calculoase
Ne-calculoase: infecii ascendente ci biliare Giardia/lamblia, malformaii ci
biliare

Macroscopic:

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/peritonit

Microscopic:

Colecistita de intensitate redus: mucoas cu exudat purulent, arii de


ulceraie ale mucoasei
Colecistita acut flegmonoas inflamaie acut n toate pturile
Colecistita gangrenoas - + tromboze vasculare

Complicaii: abces pericolecistic/peritonita

Colecistita acut

Colecistita acut

Colecistita acut

Colecistita cronic
95% asociat cu colelitiaz
Macroscopic:
Form hipertrofic
Form atrofic

Microscopic:

Inflamaie cronic cu celule mononucleate n


peretele colecistului
Fibroz parietal
Sinusuri Rokitanski-Aschoff (90%)
Metaplazie: piloric, gastric, intestinal
Foliculi limfoizi colecistit cronic
folicular

Colecistit cronic

Hipertrofia peretelui colecistului

Mucocel al veziculei biliare peretele subire, suprafaa


neted i secreia mucoas
incolor (hidrops vezicular)

Colecistita cronic

Vezicula de porelan

Tumorile veziculei biliare


Tumori benigne:
Polipul mucos:

Adenomatoi
Hiperplazici
Colesterolozici
Inflamator
Fibros

Adenomioza
Hemangiom/leiomiom/lipom/paragangliom/limfangiom

Tumori maligne:

Carcinomul de vezicul biliar


Sarcoamele
Carcinosarcoamele (tumori mixte)
Carcinoidul

Tumori secundare

Polip adenomatos
tubular/papilar

Adenomioza

Carcinomul veziculei biliare


Locul 5 n cadrul tumorilor gastro-intestinale
Frecven:

Decada 6-7
F/B-3/1
Antecedente: colelitiaz (75%)/colecistit cronic (50%)

Macroscopic:

Localizare de elecie n zona fundic, tumoare vegetant/infiltrativ

Microscopic:

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 bun
Diseminare limfatic (ganglioni regionali)/hematogen (hepatice)

Carcinomul de vezicul
biliar

Adenocarcinom

Bine difereniat

Moderat difereniat

Carcinom scuamocelular

Clasificarea OMS tumori


vezicale
Primary Tumor (T)
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1 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 liver
T3 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 structures
Regional Lymph Nodes (N)
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Metastases to nodes along the cystic duct, common bile duct, hepatic
artery, and/or portal vein
N2 Metastases to periaortic, pericaval, superior mesenteric artery, and/or
celiac artery lymph nodes

PROGNOSTIC FEATURES
In 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 FEATURES
Histologic 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 prognostici
PROGNOSTIC FACTORS (SITE-SPECIFIC FACTORS)
(Recommended for Collection)
Required for staging None
Clinically significant
Tumor location
Specimen type
Extent of liver resection
Free peritoneal side vs. hepatic side for T2

Carcinoamele cilor biliare


Inciden:

0,012-0,54% autopsii/ 0,3-1,8% operaii tract biliar

Decada 6-7
Antecedente:

Chirurgie biliar primar (20%)


Infecii: Clonorchis sinensis
Fibroza chistic familial
Colelitiaza (33%)

Macroscopic:

Tumori Klatskin (tumori hilare) fuziunea canalelor hepatice


40-55% proximale
20-25% medii
20-25% distale

Vegetante/infiltrative

Microscopic:

Adenocarcinoame 90-95%
Adenoscuamos 6,5%
Scuamocelular

Evoluie: rapid, metastaze limfatice/nu hematogene pacienii se pierd


rapid

Carcinom ci biliare

Carcinom ci biliare

Clasificarea OMS tumori ci biliare


Primary Tumor (T)
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1 Tumor con fi ned to the bile duct, with extension up to the muscle
layer or fi brous tissue
T2a 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 involvement
Regional Lymph Nodes (N)
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 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 FEATURES
Patients 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 None
Clinically significant
Tumor location
Papillary variant
Tumor growth pattern
Primary sclerosing cholangitis
CA 19-9