Extrahepatic bile ducts
Tumors
Carcinoma of extrahepatic bile ducts

Author: Hanni Gulwani, M.D.

Revised: 14 June 2018, last major update September 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Carcinoma[TI] extrahepatic bile ducts[TI]

Cite this page: Gulwani, H. Carcinoma of extrahepatic bile ducts. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/gallbladdercarcinomaextrahepatic.html. Accessed June 22nd, 2018.
Definition / general
  • Rare (1 per 100,000 in U.S.); 90 - 95% of extrahepatic bile duct malignancies are adenocarcinomas (bile duct carcinoma, cholangiocarcinoma)
  • Present in 0.5% of autopsies
  • 2 - 3 times less common than gallbladder carcinoma
Clinical features
  • More common in Native Americans, Mexicans, Israelis, Japanese
  • Present with painless, progressive jaundice; 1/3 have gallstones (10% in bile ducts themselves), 20% had prior biliary tract surgery
  • Usually age 60+; rare before age 40 unless have risk factors below
  • Small at diagnosis because even small tumors cause obstruction and jaundice

Risk factors:
  • Clonorchis sinensis and Opisthorchis viverrini infestations, primary sclerosing cholangitis, chronic ulcerative colitis, choledochal cysts, Caroli disease, congenital hepatic fibrosis
  • Also cystic fibrosis, familial polyposis coli, chronic typhoid carriers, biliary giardiasis, Thorotrast exposure, pancreaticobiliary maljunction (PBM) with bile duct dilatation (J Hepatobiliary Pancreat Surg 2008;15:15)

Spread and metastases:
  • Local extension to ampulla of Vater, colon, duodenum, gallbladder, liver, omentum, pancreas, stomach
  • Tumors from right or left hepatic duct usually extend proximally into liver or distally to common hepatic duct
  • Tumors from cystic duct extend to gallbladder or common bile duct
  • Tumors from distal common bile duct extend to pancreas, duodenum, stomach, colon, omentum
  • Metastases to regional lymph nodes, liver, lungs, peritoneum
Klatskin (hilar) tumors
  • 70% of tumors
  • Arise at confluence of right and left hepatic ducts at liver hilus
  • Slow growing with infrequent distant metastases, have marked sclerosing characteristics
  • Poorer prognosis since difficult to resect
  • 28 - 89% have positive margins
Diagnosis
  • Tissue diagnosis is optimal because clinical diagnosis is often incorrect
  • Also brushings, bile drainage cytology
Laboratory
  • Elevated alkaline phosphatase but normal serum bilirubin suggests location above hepatic duct bifurcation or incomplete common bile obstruction
Prognostic factors
  • Presence of intraepithelial spread is not an indicator of a poor prognosis but carcinoma in situ at the bile duct stump can cause recurrence (Mod Pathol 2008;21:807)
  • Mean survival 6 - 18 months, 2 years if resectable
  • 5 year survival is only 5% but is 60% for T1 tumors (which are rare)

Favorable:
Unfavorable:
Case reports
Treatment
  • Klatskin tumors require resection of hepatic duct bifurcation
  • Distal tumors may require Whipple procedure
Gross description
  • Either firm, gray nodules within bile duct wall or diffusely infiltrative (2%)
  • Often extends into adjacent structures
  • Limits of tumor often difficult to detect due to desmoplasia
  • Tumors may be papillary, multifocal and friable
Gross images

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Bile duct carcinoma

Microscopic (histologic) description
  • Nodular or diffusely infiltrative tumors with marked desmoplastic response
  • Sclerosing, nodular, polypoid papillary or mixed types
  • Resembles gallbladder carcinoma
  • Most are well or moderately differentiated with conspicuous glands but have extensive perineural invasion
  • Even well differentiated tumors may have poorly differentiated foci deep within wall
  • Mucin always present within tumor cells and glandular lumina
  • Tumor cells cuboidal or columnar, with vesicular nuclei and prominent nucleoli
  • Usually angiolymphatic invasion, necrosis and chronic inflammatory infiltrate
  • Often adjacent intestinal and pylori metaplasia
  • Dysplasia usually present
  • Variants include adenosquamous, clear cell, colloid, mucoepidermoid, small cell, squamous cell, undifferentiated (pleomorphic, sarcomatoid, giant cell) carcinomas
  • Pyloric gland phenotype involves younger patients, usually well differentiated tumors with characteristic stellar pattern, extensive perineural invasion, MUC6+ and MUC5AC+ (Hum Pathol 2012;43:2292)
  • Diagnostically difficult cases are extremely well differentiated but still have thickened duct wall with prominent desmoplastic response and perineural invasion
Microscopic (histologic) images

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Contributed by Andrey Bychkov, M.D., Ph.D., Kameda Medical Center

Adenocarcinoma



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Choledochal duct adenocarcinoma

Adenocarcinoma of the gallbladder

Molecular / cytogenetics description
Differential diagnosis