Gastrointestinal · Year 2 · from Gastrointestinal
Case 3: Cholangiocarcinoma (Klatskin Tumor)
Patient Presentation
Demographics: 65-year-old male
Chief Complaint: Painless jaundice, pruritus, and weight loss for 3 weeks
History of Present Illness: The patient noticed progressive yellowing of his skin and eyes over 3 weeks. He has intense generalized pruritus, worse at night, interfering with sleep. He has lost 12 pounds unintentionally. He denies abdominal pain but reports clay-colored stools and dark urine.
Past Medical History: Primary sclerosing cholangitis diagnosed 15 years ago, ulcerative colitis (well-controlled on mesalamine)
Social History: Never smoker, no alcohol
Physical Examination
- Vital Signs: Normal
- General: Jaundiced, cachectic male with excoriations from scratching
- HEENT: Icteric sclerae
- Abdomen: Hepatomegaly, no palpable gallbladder (unlike distal CBD obstruction), no ascites
- Skin: Jaundice, multiple scratch marks
Workup and Results
- Liver Function Tests: Total bilirubin 18.2 mg/dL, Direct 15.6 mg/dL, ALP 680 U/L, GGT 520 U/L, AST 85 U/L, ALT 92 U/L
- CA 19-9: 485 U/mL (elevated)
- MRCP: Biliary stricture at the confluence of left and right hepatic ducts with intrahepatic ductal dilation bilaterally; no single dominant mass
- CT Chest/Abdomen/Pelvis: Hilar mass with vascular encasement; no distant metastases
- ERCP with Brushings: Stricture at hepatic confluence; cytology shows adenocarcinoma
MRCP demonstrating a Klatskin tumor (perihilar cholangiocarcinoma) at the confluence of the right and left hepatic ducts. Note the dilated intrahepatic ducts bilaterally with abrupt cutoff at the hilum.
Image Source: Case courtesy of Radiopaedia.org
Diagnosis
Perihilar Cholangiocarcinoma (Klatskin Tumor) - Bismuth Type IIIA/B
Clinical Correlation
Cholangiocarcinoma arises from bile duct epithelium and is classified by location: intrahepatic, perihilar (Klatskin tumor - most common), or distal. Primary sclerosing cholangitis is the strongest risk factor, conferring 10-15% lifetime risk. Other risk factors include liver flukes, choledochal cysts, and chronic hepatitis. The Bismuth-Corlette classification stages perihilar tumors by extent of ductal involvement. Klatskin tumors present with painless obstructive jaundice; the absence of a palpable gallbladder (unlike distal tumors) reflects intrahepatic obstruction above the cystic duct insertion. CA 19-9 is elevated in most cases but lacks specificity. Prognosis is poor, with 5-year survival <20% even with resection.
Treatment
- Biliary drainage: ERCP with bilateral stent placement for jaundice palliation
- Multidisciplinary tumor board review
- Staging evaluation: EUS, consider staging laparoscopy
- If resectable: Extended hepatectomy with bile duct resection and regional lymphadenectomy (requires adequate future liver remnant)
- Selected patients: Liver transplant following neoadjuvant chemoradiation protocol (at specialized centers)
- If unresectable/metastatic: Palliative chemotherapy (gemcitabine + cisplatin)
- Pruritus management: Cholestyramine, ursodiol, consider rifampin or naltrexone
- Enhanced PSC surveillance in remaining patients with PSC (annual imaging + CA 19-9)