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    Caroli Disease and Cholangiocarcinoma

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    Danielle Foster
    This document summarizes Caroli disease and cholangiocarcinoma. Caroli disease is a congenital disorder characterized by multifocal dilation of the large intrahepatic bile ducts. It can occur alone as Caroli disease or with congenital hepatic fibrosis as Caroli syndrome. Cholangiocarcinoma is a rare type of cancer that forms in the bile ducts. Risk factors include certain liver diseases and infections. Diagnosis involves imaging tests and biopsies. Treatment options are limited as most cases are not eligible for curative surgery.

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    Caroli Disease and Cholangiocarcinoma

    Uploaded by

    Danielle Foster
    92 views10 pages
    This document summarizes Caroli disease and cholangiocarcinoma. Caroli disease is a congenital disorder characterized by multifocal dilation of the large intrahepatic bile ducts. It can occur alone as Caroli disease or with congenital hepatic fibrosis as Caroli syndrome. Cholangiocarcinoma is a rare type of cancer that forms in the bile ducts. Risk factors include certain liver diseases and infections. Diagnosis involves imaging tests and biopsies. Treatment options are limited as most cases are not eligible for curative surgery.

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    caroli disease and cholangiocarcinoma pathology

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    This document summarizes Caroli disease and cholangiocarcinoma. Caroli disease is a congenital disorder characterized by multifocal dilation of the large intrahepatic bile ducts. It can occur alone as Caroli disease or with congenital hepatic fibrosis as Caroli syndrome. Cholangiocarcinoma is a rare type of cancer that forms in the bile ducts. Risk factors include certain liver diseases and infections. Diagnosis involves imaging tests and biopsies. Treatment options are limited as most cases are not eligible for curative surgery.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    92 views10 pages

    Caroli Disease and Cholangiocarcinoma

    Uploaded by

    Danielle Foster
    This document summarizes Caroli disease and cholangiocarcinoma. Caroli disease is a congenital disorder characterized by multifocal dilation of the large intrahepatic bile ducts. It can occur alone as Caroli disease or with congenital hepatic fibrosis as Caroli syndrome. Cholangiocarcinoma is a rare type of cancer that forms in the bile ducts. Risk factors include certain liver diseases and infections. Diagnosis involves imaging tests and biopsies. Treatment options are limited as most cases are not eligible for curative surgery.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 10

    Caroli Disease &

    Colangiosarcoma
    Table of Contents
    Caroli Disease
    Pathogenesisi
    Clinical manifestations
    Differential diagnosis
    Cholangiosarcoma
    Caroli Disease &
    Syndrome
    Congenital disorder
    characterized by
    multifocal segmental
    dilation of large
    intrahepatic bile ducts
    Caroli disease
    bile ductular extasia
    without other apparent
    hepatic abnormalities
    Segmental dilation of large
    intrahepatic biliary ducts
    Caroli syndrome
    Segmental dilation of small
    intra-hepatic biliary ducts &
    congenital hepatic fibrosis
    ETIOLOGY
    ARPKD gene for fibrocystin
    linked to caroli syndrome
    Pathophysiology
    Biliary tree develops from Ductal plate
    Caroli Disease
    Abnormalities of bile duct at large intrahepatic
    ducts- dilation and ectasia
    Biliary stasis can lead to cholelithiasis, cholangitis,
    sepsis, and increased risk of cholangiocarcinoma
    Caroli Syndrome
    Ductal plate malformation at smallet portal racts-
    varying degrees of portal fibrosis
    Caroli Disease &
    Syndrome
    Generally children (as it is congenital)
    Very rare: disease is 1:100,000 births, Syndrome
    1: 20,000
    F>M
    Present with history of intermittent abdominal
    pain, RUQ pain, fever or family history of ARPKD
    Differential Diagnosis:
    Congenital hepatic fibrosis
    Pediatric gallstones
    Primary sclerosing cholangitis
    Increased risk of developing cholangiocarcinoma
    Cholangiocarcinoma
    Malignancies of
    biliary duct
    system
    Origginate in liver
    and extrahepatic
    bile ducts
    Intrahepatic
    Extrahepatic
    (perihilar)
    Distal
    extrahepatic
    Cholangiocarcinoma
    2500 new cases
    Grow slowly and each year
    infiltrate walls of 2-6:100,000 in
    ducts western
    Local extension: countries
    liver, porta 5.5:1000,000
    hepatitis, Japan,
    regional lymph 7.3:100,000
    nodes (celiac and Israel
    pancreaticoduod Most commonly
    enal change) women older
    than 60 years
    Risk Factors & Diagnosis:
    Cholangiocarcinoma
    ParasiticLiver Diseases Diagnosis
    Opisthorchis viverrini
    Clonorchis sinensis Bippsy
    Vrial Liver Disease Blood tests: CA
    Cirrhosis
    19-9
    HIV infection
    Congenital Liver
    Abdominal
    abnormalities Imaging (CT and
    Hepatolithiasis ultrasound
    Thorotrast exposure Endoscopic
    IBD
    Retrograde
    Alpha1-antitrypsin
    deficiency
    Cholangiopancrea
    obesity tiography
    Cholangiocarcinoma
    History
    Jaundice 90% of patients
    Clay colored are not eligibale
    stools for curative
    Bilirubinuria resection
    Pruritis
    Survival is
    Weight loss
    approximately 6
    Abdominal pain
    months
    +Courvoisier
    sign
    Morphology

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