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Cholangiocarcinoma

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megan horner

on 14 August 2012

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Transcript of Cholangiocarcinoma

Cholangiocarcinoma
symptomatic when bile duct becomes compressed and bile flow is blocked
Symptoms
pruritis
weight loss - 1/3 of patients
vague RUQ pain
fever
acholic stools
dark urine - bilirubinuria
DDx
choledocholithiasis
cholelithiasis
choledochal cysts
cholangitis
strictures in biliary system
PSC
pancreatic head mass
Imaging
abdominal US or CT scan first
Followed by cholangiography
MRCP, ERCP
PET scan and angiography
ABOUT
PRESENTATION
Cancer arising from bile duct epithelial cells
highly lethal due to late presentation
includes the intrahepatic ducts and extrahepatic ducts
tumor at the confluence of R and L hepatic ducts = Klatskin Tumor = 70% of cholangiocarcinomas
Primary sclerosing cholangitis is a risk factor
Labs
direct hyperbilirubinemia
elevated alkaline phosphatase
5' nucleotidase and GGT
ALT/AST elevate with chronic biliary obstruction
DIAGNOSIS
Tumor markers
CEA
CA 19-9
neither sensitive nor specific
mostly used to monitor treatment, detect recurrence and in pts. with PSC
MAKING A TISSUE DIAGNOSIS IS DIFFICULT
CASE
HPI: 63 yo Hispanic female presents with
painless jaundice for the past 3 months. C/o extreme pruritis, fatigue, 14 lb. weight loss, and some night sweats. She reported to her dermatologist for pruritis where she was prescribed topical steroid cream with no relief.

Associated Sx: early satiety and epigastric discomfort

PSH: cholecystectomy 20+ years ago

Social: + 30 pack year history of smoking

Meds: None except steroid cream
CASE
PE: afebrile, scleral icterus, jaundice, TTP epigastrum and RUQ
DDx:
Pancreatic head mass
primary bile duct stone
cholangiocarcinoma
primary biliary cirrhosis
CASE
Labs:
WBC: 16.9
T/D Bili: 15.9/13.0
ap: 378
GGT: 103
AST/ALT: 70/61
UA: + bilirubin
1. CT abd/pelvis - no obvious masses
2. ERCP - stricturing at the confluence of the L and R hepatic ducts, placement of stents x2, sphincterotomy, cytology brushings @ stricture
worrisome for Klatskin Tumor
Imaging
no cholangiographic features specific for cholangiocarcinoma
worrisome features include
irregular narrowing of duct
polypoid lesions
rapidly progressing strictures
cytology brushings sensitivity of only 30-85%
difficult to detect extraductal reach of the tumor
Preoperative Decompression
stent placement at location of stricture
highly debatable
may obscure later assessment of resectability
Resectability
depends on location and extent of disease
difficult to assess on imaging
many times must assess intraoperatively
signs of unresectability:
+ nodes outside of hepatic pedicle
distant metastasis
bilateral extension into secondary hepatic ducts
bilateral extension into hepatic parenchyma
combined vascular invasion and contralateral ductal spread
Surgical Options
Distal extrahepatic:
Whipple procedure (pancreaticoduodenectomy)
Intrahepatic:
hepatic resection (caudate lobe) to achieve negative margins +/- portal node dissection
Perihilar (Klatskin's)
surgery type depends on Bismuth-Corlette classification and now T-staging
type I and II:
en bloc resection extrahepatic ducts with margins, regional lymphadenectomy, roux-en-y hepaticojejunostomy
type III:
above plus caudate lobectomy
Type IV:
portal vein resection

most common recurrence is local due to early involvement of caudate lobe ducts
whipple's procedure for
distal cholangiocarcinoma
roux-en-y hepaticojejunostomy for
hilar cholangiocarcinoma
Prognosis
Adjuvant therapy
insufficient trials to prove benefit
most receive combined chemo/rads
to prevent local recurrence
possible synergistic effect of chemo/rads on cells
controversy over current prognosis
90% not candidates for curative resection
5 year survival rate is typically 5-10 %
some studies are showing improving 5 year mortality rates of up to 50% due to partial hepatectomy (caudate lobe) during local resection
partial hepatectomy added to achieve negative margins
prognostic factors include:
margin status
vascular invasion
lymph node metastasis
References
Lowe, R. C., Afdhal, N. H., & Anderson, C. D. (2012). Clinical manifestations and diagnosis of cholangiocarcinoma . UpToDate, Retrieved from http://www.uptodate.com.ezproxy.dmu.edu:2048/contents/clinical-manifestations-and-diagnosis-of-cholangiocarcinoma?source=see_link

Anderson, C. D., & Stuart, K. E. (2011). Treatment of localized cholangiocarcinoma: Surgical management and adjuvant therapy . UpToDate, Retrieved from http://www.uptodate.com.ezproxy.dmu.edu:2048/contents/treatment-of-localized-cholangiocarcinoma-surgical-management-and-adjuvant-therapy?source=search_result&search=klatskin tumor&selectedTitle=2~12

Radiologic Manifestations of Sclerosing Cholangitis with Emphasis on MR Cholangiopancreatography
Radiographics July 2000 20:959-975
Intrahepatic
Extrahepatic
Perihilar**
Distal Extrahepatic
Types
95% adenocarcinoma
Native Americans highest incidence - chronic parasite infection

Highest prevalence in 6th and 7th decades of life
Physical signs
painless jaundice- location dependent
hepatomegaly
RUQ mass
Courvosier's sign- distal to cystic duct
Epidemiology
Risk Factors
Parasites - Chlonorchis sinensis and Opisthorchis viverrini
PSC
Ulcerative Colitis
chemical exposure
Staging
TNM system

Perihilar
Bismuth Corlette- longitudinal spread
Blumgart T-staging - vascular invasion and hepatic lobe atrophy
better for selecting surgical candidates and predicting survival
Megan Tasker PGY-2
General Surgery
Question:
Which of the following is a contraindication to resection of an adenocarcinoma of the bile duct?

a. Tumor location in distal CBD
b. Tumor location in bile duct bifurcation
c. Peritoneal mets
d. Invasion of R portal vein & hepatic artery
e. None of the above
Answer
C. peritoneal metastasis
prognosis related to tumor location, resectability & histologic pattern
proximal lesions more common but poorest prognosis due to being least resectable
aggressive resection of proximal lesions can improve survival
unresectable if mets, b/l involvement of portal v or hepatic a., extension to secondary bile ducts
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