Klatskin tumor
Formal Definition
Klatskin tumor — a cholangiocarcinoma arising at the confluence of the right and left hepatic ducts within the porta hepatis (the bifurcation of the common hepatic duct into right and left branches), classified as a perihilar cholangiocarcinoma (Bismuth-Corlette classification type I–IV); presents with painless obstructive jaundice, pruritus, weight loss, and often cholangitis if bacterial superinfection occurs; tumor biology tends to be slow-growing and locally invasive but prognosis remains poor because most patients present at advanced stage when the tumor encases portal vein, hepatic artery, or contralateral hepatic duct.
How It's Used on the Ward
"Klatskin" — the type of bile-duct cancer at the bifurcation/cloison where the right and left hepatic ducts meet; presents as painless jaundice with weight loss; anatomically tricky because it strands the entire liver drainage; treated surgically when possible with bile-duct resection ± hepatic lobectomy, or palliated with stenting.
Example
""68-year-old previously healthy woman presents with 6 weeks of painless jaundice, pruritus, fatigue, and 8-pound weight loss. Exam: scleral icterus, no palpable RUQ mass, no lymphadenopathy. Labs: bilirubin 12.4 (mostly direct), alk phos 720, mild transaminitis (AST 88, ALT 102), CA 19-9 480. Imaging: MRI/MRCP shows tumor at the hepatic duct confluence with proximal biliary dilation in both lobes; no portal vein invasion on dedicated protocol. Diagnosis: Klatskin tumor (Bismuth IIIa — extends to right hepatic duct). Multidisciplinary review: surgical candidate — extended right hepatectomy with hepaticojejunostomy. Pathology after resection confirms R0 margins; adjuvant capecitabine planned.""
Clinical Context
Bismuth-Corlette classification: Type I — common hepatic duct, distal to bifurcation. Type II — involves bifurcation, does not extend into right or left hepatic ducts. Type IIIa — extends into right hepatic duct. Type IIIb — extends into left hepatic duct. Type IV — involves both right and left hepatic ducts (multicentric). Clinical presentation: painless jaundice (unlike pancreatic cancer pain, often silent), pruritus (from bile salt accumulation), dark urine, pale stools, fatigue, weight loss, cholangitis if biliary obstruction becomes infected. Workup: MRI/MRCP (best for biliary anatomy), contrast-enhanced CT for resectability staging, endoscopic ultrasound for biopsy, ERCP with brushings for cytology (often non-diagnostic), CA 19-9 elevation (helpful but not specific). Staging: many systems; key factors include vascular involvement (portal vein, hepatic artery), contralateral ductal extension, lobar atrophy, distant metastases. Treatment: surgical resection (only curative option; requires experienced hepatobiliary center), liver transplantation (selected cases meeting Mayo protocol criteria), palliative biliary drainage (stenting for jaundice), systemic chemotherapy (gemcitabine + cisplatin historically; durvalumab + chemo more recently per TOPAZ-1 trial). Prognosis: 5-year survival 30-50% for resected early Bismuth I-II; <10% for advanced unresectable disease.