Adenocarcinoma of the Extrahepatic Bile Ducts



Adenocarcinoma of the Extrahepatic Bile Ducts


Hanlin L. Wang, MD, PhD









Segmental resection for períhilar bile duct carcinoma shows marked thickening of the common hepatic duct with firm, white cut surfaces image. A portion of dilated cystic duct is present image.






Cross section of a Whipple specimen shows distal bile duct carcinoma involving the intrapancreatic portion of the common bile duct and causing marked thickening of the duct wall image.


TERMINOLOGY


Synonyms



  • Extrahepatic cholangiocarcinoma


Definitions



  • Malignant neoplasm arising from epithelium lining right and left hepatic ducts, common hepatic duct, and common bile duct


  • Perihilar bile duct carcinoma



    • Arises in extrahepatic bile ducts upstream to origin of cystic duct



      • Klatskin tumor occurs at confluence of right and left hepatic ducts


    • Comprises 70-80% of extrahepatic cholangiocarcinoma


  • Distal bile duct carcinoma



    • Arises in common bile duct (including intrapancreatic portion) above ampulla of Vater


    • Comprises 20-30% of extrahepatic cholangiocarcinoma


  • Diffuse involvement of extrahepatic bile ducts is rare, comprising ˜ 2% of extrahepatic cholangiocarcinoma


ETIOLOGY/PATHOGENESIS


Developmental Anomaly



  • Choledochal cyst


  • Abnormal choledochopancreatic junction


Chronic Inflammation



  • Primary sclerosing cholangitis


  • Cholelithiasis (controversial)


Parasitic Infection (Flukes)



  • Clonorchis sinensis


  • Opisthorchis viverrini


Genetic Syndromes



  • Familial adenomatous polyposis


Molecular Alterations



  • KRAS mutations in ˜ 30% of cases


  • Overexpression of p53 oncoprotein in ˜ 50% of cases


CLINICAL ISSUES


Epidemiology



  • Incidence



    • 0.53-2 per 100,000 in population


  • Age



    • Primarily seen during 6th and 7th decades of life


  • Gender



    • Slight male predominance


Presentation



  • Nonspecific symptoms and signs



    • Abdominal pain, malaise, anorexia, nausea, vomiting, weight loss


  • Symptoms and signs of biliary obstruction



    • Jaundice, pruritus, acholic stools, dark urine


Laboratory Tests



  • Elevated serum CA19-9, CEA, and CA125 levels


Treatment



  • Surgical resection



    • Only hope for long-term survival


    • Segmental resection



      • May include partial hepatectomy for perihilar bile duct carcinoma


    • Whipple procedure for distal bile duct carcinoma


  • Combined modality therapy, including chemotherapy and radiotherapy


Prognosis



  • 10% overall 5-year survival


  • Prognostic indicators



    • Tumor stage



      • Most important prognostic indicator


    • Tumor location




      • Better prognosis for distal bile duct carcinoma due to early detection and resectability


    • Histologic grade



      • Poorly differentiated tumors are associated with worse prognosis


    • Histologic variants



      • Poorer prognosis for signet ring cell carcinoma


      • More favorable outcome for papillary adenocarcinoma


    • Surgical resection margins



      • Improved overall survival for those with negative resection margins


    • Lymphovascular invasion



      • Associated with adverse outcome


    • Perineural invasion



      • Associated with adverse outcome


IMAGE FINDINGS


Ultrasonographic Findings



  • Duct dilation indicative of downstream obstruction


CT and MR Findings



  • Infiltrative pattern



    • Duct wall thickening, obliteration of duct lumen


  • Mass-like lesion



    • Distension of duct by intraluminal mass


Cholangiographic (ERCP) Findings



  • Bile duct stricture


MACROSCOPIC FEATURES


General Features



  • Firm, white, and gritty cut surface


  • 4 categories traditionally



    • Polypoid


    • Nodular


    • Scirrhous constricting


    • Diffusely infiltrating


MICROSCOPIC PATHOLOGY


Histologic Features

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Adenocarcinoma of the Extrahepatic Bile Ducts
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