Tumors of the Biliary Tract

Key Points

1
Cancers of the biliary tract are categorized into four groups: Gallbladder cancer, intrahepatic cholangiocarcinoma, perihilar cholangiocarcinoma, and distal cholangiocarcinoma.
2
Disregarding cases discovered incidentally at cholecystectomy, carcinoma of the gallbladder, because of its late stage of clinical presentation, has an overall 5-year survival rate <20%.
3
Surgical resection that leads to negative margins may be curative for patients with localized cancers limited to the gallbladder wall.
4
Cholangiocarcinoma is strongly associated with cystic disease of the biliary tract (choledochal cysts, Caroli disease), parasitic infection ( Clonorchis sinensis or Opisthorchis viverrini ), primary sclerosing cholangitis, and hepatolithiasis.
5
Patients with perihilar cholangiocarcinomas have poor overall survival, although aggressive resection that includes hepatectomy to achieve negative margins may provide cure.
6
Distal bile duct tumors, which manifest similarly to other periampullary malignant tumors, are associated with a higher rate of resectability and better long-term survival than more proximal (hilar and intrahepatic) cholangiocarcinomas.

1.
The most commonly observed polypoid lesion of the gallbladder; accounts for approximately 50% of such lesions
2.
Not true neoplasms, but rather cholesterol-filled projections of gallbladder mucosa protruding into the lumen
3.
Usually <1 cm in size; visualized on gallbladder imaging studies (ultrasonography, oral cholecystography) as nonmobile filling defects
4.
Usually asymptomatic unless associated with gallstones or chronic cholecystitis (e.g., porcelain gallbladder [see later and Chapter 35 ])
5.
No malignant potential

1.
Consists of a thickened gallbladder muscular layer with Rokitansky-Aschoff sinuses
2.
Three types: Fundal (most common), appearing as a hemispheric lesion with a central dimple; segmental, consisting of an annular stricture; or diffuse, involving the entire gallbladder
3.
May manifest as muscular hypertrophy secondary to gallbladder dysmotility; therefore, symptoms are relieved by cholecystectomy
4.
May be associated with carcinoma of the gallbladder

1.
True neoplastic epithelial tumors of the gallbladder mucosa
2.
Usually manifest as solitary, nonmobile filling defects seen on gallbladder ultrasonography
3.
Premalignant, with carcinoma in situ found in larger polyps
4.
Unlikely to play a major role in the pathogenesis of most gallbladder cancers

1.
Because the histology of polypoid lesions of the gallbladder cannot be determined nonoperatively by current methods, even high-quality ultrasonography, patients with polyps >8 mm should undergo cholecystectomy.
2.
Polyps up to 8 mm in size, regardless of total number, should be followed by repeat imaging studies every 3 to 6 months. Changes in ultrasonographic size or features (e.g., involvement of the gallbladder wall) are indications for cholecystectomy.
3.
Any patient with biliary symptoms and a gallbladder polyp should undergo cholecystectomy.

1.
Much less common than benign gallbladder tumors
2.
Histologic types
- ▪
  Papillomas
- ▪
  Adenomas
- ▪
  Cystadenomas: Tumors with inner layers of mucin-secreting epithelium, mesenchymal stroma, and an outer layer of hyalinized fibrous tissue
- ▪
  Other lesions that may mimic a malignancy
  - –
    Benign tumors: Soft tissue sarcoma, neuroendocrine tumors, and neuromas
  - –
    Inflammatory conditions: Viral hepatitis infection, cholangiopathy due to immunoglobulin (Ig)G4–related disease, biliary sclerosis, radiation-induced cholangiopathy, human immunodeficiency virus (HIV)–associated cholangiopathy (see Chapter 27 ), and iatrogenic injuries postcholecystectomy of the extrahepatic biliary tract
3.
They may be solitary or multiple.
4.
Symptoms are usually caused by bile duct obstruction, which results in intermittent jaundice or cholangitis.
5.
The diagnosis can usually be made by magnetic resonance, endoscopic retrograde, or percutaneous transhepatic cholangiography.
6.
Treatment consists of surgical resection of the bile duct, most commonly with reconstruction by hepaticojejunostomy.
7.
Both benign cystadenomas and multiple papillomatosis of the bile duct can be associated with a high rate of local recurrence if complete resection is not accomplished.

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