AKT · Gastroenterology/Pancreatobiliarylow yield

Cholangiocarcinoma

Adenocarcinoma of the bile-duct epithelium (PSC, liver flukes, choledochal cysts)

Overview

Malignancy of the bile-duct epithelium (intra-hepatic, perihilar/Klatskin, or distal). Presents like other malignant biliary obstruction - PAINLESS obstructive jaundice, weight loss, RUQ pain - often late and unresectable. Risk factors: primary sclerosing cholangitis, liver flukes, choledochal cysts, chronic biliary inflammation. CA19-9 raised; diagnosed on imaging/cytology; only a minority are resectable.

Recognise

PAINLESS obstructive JAUNDICE (dark urine, pale stools, itch), weight loss, RUQ pain; +/- palpable gallbladder (Courvoisier) for distal tumours
Cholestatic LFTs, raised CA19-9; a dominant stricture/mass on imaging; Klatskin tumour at the hilum
Risk factors: PSC, liver flukes (Clonorchis/Opisthorchis), choledochal cysts, hepatolithiasis

Red flags

Malignant biliary obstruction with cholangitis -> decompression + antibiotics
New dominant stricture in PSC -> cholangiocarcinoma until excluded

Differentials & how to tell them apart

Pancreatic head cancerboth cause painless obstructive jaundice - imaging localises (pancreatic mass vs biliary stricture)

Choledocholithiasisusually PAINFUL +/- cholangitis; stone not stricture/mass

PSC dominant stricture (benign)brushings/cytology to distinguish benign from malignant in PSC

Investigations

Cholestatic LFTs, CA19-9; USS then CT/MRI/MRCP (stricture/mass, staging); ERCP/cholangioscopy with brushings/biopsy; exclude/treat obstruction.

Management

Resection if possible (minority); biliary stenting + chemotherapy if not

1Image and obtain tissue (brushings/biopsy); relieve obstruction (stent). Assess resectability.Gate: A new dominant stricture in PSC must be assumed to be cholangiocarcinoma until excluded; distinguish from pancreatic cancer (imaging) and benign stone disease (painful, no mass)
2Resectable (few) -> surgery + adjuvant chemo; unresectable -> biliary stenting + palliative chemotherapy.

Surgical resection (the only curative option) — minority resectable; Whipple for distal, hepatectomy for intrahepatic/hilar

Biliary stenting (ERCP/PTC) for jaundice; chemotherapy — palliation/adjuvant

Key points

Painless obstructive jaundice + weight loss + biliary stricture/mass + raised CA19-9 = cholangiocarcinoma; PSC and liver flukes are the classic risks. Pancreatic cancer is the main mimic - imaging localises it.

Monitor & prognosis

CA19-9; biliary patency; staging.

Often late/poor; better if resectable.

Source: BSG; NICE