eMedicine Specialties > General Surgery > Abdomen

Bile Duct Tumors: Workup

Author: Todd A Nickloes, DO, Assistant Professor of Surgery, Division of Trauma/Critical Care, University of Tennessee Medical Center
Coauthor(s): Brian Reed, MD, Staff Physician, Department of Surgery, University of Tennessee Medical Center; LaMar O Mack, MD, Staff Physician, Department of Surgery, University of Tennessee Medical Center; Ravi Pokala Kiran, MBBS, MS, FRCS (Eng), FRCS (Glas), Staff Physician, Department of General Surgery, St Mary's Hospital; Naveen Pokala, MBBS, MS, FRCS, Staff Physician, Department of Surgery, Bronx Lebanon Hospital
Contributor Information and Disclosures

Updated: Mar 13, 2009

Workup

Laboratory Studies

  • Liver function tests
    • Results of liver function tests are suggestive of cholestasis in patients with bile duct tumors. Fluctuations in serum levels reflect incomplete obstruction or primary involvement of only 1 hepatic duct.
    • In complete obstruction, serum bilirubin is markedly elevated. Serum alkaline phosphatase and gamma glutamyl transferase also are markedly elevated because they are markers of bile duct injury.
    • Serum aspartate aminotransferase and alanine aminotransferase, which mark hepatocellular damage, usually are only mildly elevated.
  • Complete blood picture
    • Patients usually are anemic.
    • The leukocyte count may be high normal, with a preponderance of polymorphs.
  • Levels of serum CEA and alpha fetoprotein (AFP) usually are normal and not elevated.
  • A serum mitochondrial antibody test also produces negative results.
  • Feces are pale and fatty, with occasional blood.

Imaging Studies

  • Ultrasonographic scanning of the liver is the investigation of first choice in patients with obstructive jaundice; these scans usually reveal dilated intrahepatic biliary ducts.1 The extrahepatic duct may be collapsed if the tumor is high (eg, a Klatskin tumor at the bifurcation).
    • A tumor mass may be observed in 40% of cases as a hyperechoic lesion.
    • The absence of dilatation of intrahepatic bile ducts suggests an alternate diagnosis, such as drug-related jaundice and primary biliary cirrhosis.
  • CT scans of the abdomen demonstrate intrahepatic biliary dilatation and lobar atrophy, but tumor mass may be difficult to demonstrate.6 Calcification may be observed. CT scanning is useful in diagnosing the level of obstruction in nearly all patients, and a specific diagnosis is possible in 78% of patients.
  • Spiral CT scanning allows accurate analysis of the relationship between vascular and bile duct anatomy at the hilum.
  • Magnetic resonance imaging (MRI) of the abdomen may be performed, but it adds little to ultrasonographic and CT scanning in establishing the diagnosis of extrahepatic cholangiocarcinoma.7
  • MRI cholangiography may be valuable and is increasingly available in specialized centers.8,9,10
  • Digital subtraction angiography (DSA) is useful in the preoperative assessment of resectability and demonstrates the anatomy of the hepatic artery and portal vein.
  • Cholangiography is indicated in any patient who is cholestatic with nondilated bile ducts when the diagnosis is in doubt. The choice of cholangiographic investigation depends on the site of the tumor.
    • In proximal lesions, percutaneous transhepatic cholangiography defines the extent of the tumor and allows for the preoperative placement of percutaneous catheters.
    • Endoscopic retrograde cholangiopancreatography (ERCP) is of greater value in the diagnosis of distal tumors and permits the placement of endoprostheses (see image below and Image 5).11
  • Radiologically, cholangiocarcinomas present in 3 distinct patterns.
    • An intrahepatic mass is observed in 20-30% of cases. Calcification may be present. Ultrasonography reveals a hypoechoic, hyperechoic, or mixed echogenicity mass, whereas CT scan reveals a low-density, heterogeneous, and often peripherally enhancing mass.
    • A hilar Klatskin tumor is the most common. Ultrasonographic and CT scans of the abdomen show intrahepatic biliary dilatation with a normal-appearing cystic duct and hilar mass. In addition, segmental or lobar atrophy may exist. Portal and retroperitoneal adenopathy also are common. Cholangiography is diagnostic, with a stricture observed straddling the bifurcation and isolated right and left systems.
    • Distal duct form presents as a stricture and (less commonly) as a polypoid-filling defect. The stricture may be irregular, with overhanging edges suggestive of a malignant stricture, or it may be smooth and indistinguishable from benign strictures.

Endoscopic retrograde cholangiopancreatography (E...

Endoscopic retrograde cholangiopancreatography (ERCP) shows a narrowed area in the distal common bile duct with dilatation of the proximal biliary tree.

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Endoscopic retrograde cholangiopancreatography (E...

Endoscopic retrograde cholangiopancreatography (ERCP) shows a narrowed area in the distal common bile duct with dilatation of the proximal biliary tree.


Diagnostic Procedures

  • Tissue diagnosis can be obtained by means of percutaneous fine-needle aspiration biopsy, brush and scrape biopsy, or cytologic examination of bile.12,13 However, these techniques provide a definitive diagnosis in only 30-50% of patients. If surgery is contemplated, preoperative tissue diagnosis is not essential, and surgical exploration is indicated.

Histologic Findings

A bile duct tumor is typically quite small (see image below and Image 2); 95% are adenocarcinomas of varying differentiation. Grossly, the tumor may be nodular or papillary or may present as a stricture (scirrhous variety).

Distal common bile duct tumor excised by radical ...

Distal common bile duct tumor excised by radical pancreaticoduodenectomy. The tumor measured 1.2 cm in diameter.

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Distal common bile duct tumor excised by radical ...

Distal common bile duct tumor excised by radical pancreaticoduodenectomy. The tumor measured 1.2 cm in diameter.


Nodular bile duct tumors form extraductal nodules in addition to intraluminal projections. The papillary variety most commonly is found in the distal bile duct and may fill the duct lumen with friable, vascular neoplastic tissue, which may cause hemobilia. The scirrhous variety usually is confined to the hilar area and forms gray, annular thickenings with clear defined edges. Distinguishing this variant from sclerosing cholangitis occasionally is difficult, even on histologic grounds.

Microscopically, the tumor usually is a mucin-secreting adenocarcinoma with a cuboidal or columnar epithelium, and spread along neural sheaths may be noted.12 The scirrhous variety is intensely fibrotic and relatively acellular, often with a few well-differentiated ductal cancer cells grouped as acini in a dense connective-tissue stroma. Rare types include squamous cell carcinoma, adenosquamous carcinoma, adenoacanthoma, mucoepidermoid carcinoma, cystadenocarcinoma, granular cell carcinoma, lymphoma, carcinoid tumors, and melanoma. Malignant smooth muscle tumors of the bile duct and nonsecreting apudomas of the hilar region also have been reported.

Immunohistochemistry and molecular biological studies show that in addition to CEA, many tumors also stain positively for the carbohydrate antigens CA 50 and CA 19-9. Some reports also have identified mutations in K-ras oncogenes in 60-70% of intrahepatic and perihilar bile duct cancers. Further studies have identified abnormalities on chromosomes 5 and 17 and have documented the presence of c-erb oncogenes, epidermal growth factors, and proliferating nuclear antigens.4,14

The diagnosis of bile duct cancers may be supported by positive findings in 2 of the 3 indicators, which are a positive reaction to CEA, nuclear size variation, and the formation of distended intracytoplasmic lumina. Neural invasion is another histologic finding that confirms a diagnosis of bile duct cancer.

Staging

Staging of bile duct tumors is performed by the (primary) tumor, (regional lymph) node, (remote) metastases (TNM) system of classification.

  • T1 - Tumor limited to the mucosa or muscle layer
  • T2 - Tumor invading the periductal tissue
  • T3 - Tumor invading the adjacent structures
  • N0 - No nodal involvement
  • N2 - Involvement of regional nodes
  • M0 - No distant metastases
  • M1 - Presence of distant metastases

Stages are as follows:

  • Stage I - T1, N0, MO
  • Stage II - T2, N0, M0
  • Stage III - T1-2, N1, M0
  • Stage IVA - T3, N0-1, M0
  • Stage IVB - T1-3, N0-1, M1

More on Bile Duct Tumors

Overview: Bile Duct Tumors
Workup: Bile Duct Tumors
Treatment: Bile Duct Tumors
Follow-up: Bile Duct Tumors
Multimedia: Bile Duct Tumors
References
Further Reading
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Keywords

bile duct tumors, bile, bile duct, liver, pancreas, pancreatic, gall bladder, gallbladder, biliary, hepatic, cholecystectomy, cholangiocarcinoma, bile duct cancer, liver bile, biliary tree, biliary duct, biliary disease, gall bladder disease, gallbladder disease, gall bladder cancer, gallbladder cancer, bile duct symptoms, bile ducts, Klatskin tumor, cholangiocarcinoma of the hepatic duct bifurcation

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Contributor Information and Disclosures

Author

Todd A Nickloes, DO, Assistant Professor of Surgery, Division of Trauma/Critical Care, University of Tennessee Medical Center
Todd A Nickloes, DO is a member of the following medical societies: American College of Osteopathic Surgeons, American Medical Association, American Osteopathic Association, Association for Academic Surgery, Eastern Association for the Surgery of Trauma, Society of Critical Care Medicine, Society of Laparoendoscopic Surgeons, Southeastern Surgical Congress, and Southern Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Brian Reed, MD, Staff Physician, Department of Surgery, University of Tennessee Medical Center
Brian Reed, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, and American Medical Association
Disclosure: Nothing to disclose.

LaMar O Mack, MD, Staff Physician, Department of Surgery, University of Tennessee Medical Center
LaMar O Mack, MD is a member of the following medical societies: American Urological Association, National Medical Association, and Student National Medical Association
Disclosure: Nothing to disclose.

Ravi Pokala Kiran, MBBS, MS, FRCS (Eng), FRCS (Glas), Staff Physician, Department of General Surgery, St Mary's Hospital
Disclosure: Nothing to disclose.

Naveen Pokala, MBBS, MS, FRCS, Staff Physician, Department of Surgery, Bronx Lebanon Hospital
Disclosure: Nothing to disclose.

Medical Editor

Marc D Basson, MD, PhD, MBA, Professor, Chair, Department of Surgery, Michigan State University
Marc D Basson, MD, PhD, MBA is a member of the following medical societies: American College of Surgeons and American Gastroenterological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael A Grosso, MD, Consulting Staff, Department of Cardiothoracic Surgery, St Francis Hospital
Michael A Grosso, MD is a member of the following medical societies: American College of Surgeons, Society of Thoracic Surgeons, and Society of University Surgeons
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

John Geibel, MD, DSc, MA, Vice Chairman, Professor, Department of Surgery, Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital
John Geibel, MD, DSc, MA is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, and Society for Surgery of the Alimentary Tract
Disclosure: AMGEN Royalty Other

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