Todd M. Blodgett, MD

Alex Ryan, MD

Omar Almusa, MD

Graphic shows a typical Klatskin tumor image, which is cholangiocarcinoma near the bifurcation of the main right and left intrahepatic bile ducts.

Coronal PET (A), axial CT (B) and fused PET/CT (C) show focal intense activity in the portacaval region image of this patient with a history of cholangiocarcinoma.


Abbreviations and Synonyms

  • Cholangiocarcinoma (CC), Klatskin tumor, malignant bile duct tumor


  • Malignancy that arises from ductular epithelium of intrahepatic biliary tree and extrahepatic bile ducts

    • Note: Gallbladder cancer 9x more common than CC

  • Klatskin tumor: Perihilar cholangiocarcinoma involving bifurcation of hepatic duct; accounts for more than 70% of all bile duct cancers


General Features

  • Best diagnostic clue

    • PET: Hypermetabolic activity corresponding to primary tumor in liver, extrahepatic metastatic disease

    • Ultrasound, CT, MR: Bile duct obstruction w/small central mass suggests hilar lesion (Klatskin tumor)

  • Location

    • Extrahepatic tumors (87-92% of CC): Proximal, middle, distal ductal tumors

    • Extrahepatic tumor at bifurcation of proximal common hepatic duct = Klatskin tumor

    • Intrahepatic tumors (8-13% of CC) arise from small ducts

    • Nodular or papillary type is most common in distal duct and periampullary region

    • Intrahepatic tumors have tendency for perineural spread, but spread to liver, peritoneum, lung is extremely rare

    • Extrahepatic tumors spread to celiac nodes in ˜ 16% of cases

  • Size

    • Peripheral lesions are usually larger, measuring 5-20 cm at presentation

    • More central lesions (Klatskin) smaller at diagnosis

  • Morphology

    • Variable

    • Most intrahepatic CC present as mass, whereas 90% of extrahepatic CC reveal diffusely infiltrating growth pattern

Imaging Recommendations

  • Best imaging tool

    • CT: Staging regional/distant metastases; similar to US for demonstrating ductal dilation, large mass lesions

    • MRCP/ERCP: Sensitivity of 71-81% for detecting tumor in malignant stenoses, particularly central lesions

    • PET for staging distant metastases and characterizing peripheral CC

    • ERCP with brush cytology, DNA analysis, and serum analysis of CA 19-9 and CEA for initial workup

      • Have been shown to increase sensitivity significantly

      • Diagnosis of CC, especially in primary sclerosing cholangitis (PSC), may remain uncertain until invasive and aggressive approaches such as exploratory laparotomy provide biopsy

  • Protocol advice

    • Delayed PET imaging at ˜ 120 minute time point shown to better discriminate tumor from inflammation

    • Delayed imaging helps differentiate tumor from background liver activity

CT Findings

  • NECT

    • Mass predominantly hypoattenuating with irregular margins

    • Intrahepatic biliary duct (IHBD) dilation common with obstruction

    • Larger peripheral lesions may be isodense with central low attenuation and scarring

      • Central and satellite lesions

    • Hilar masses often not visible on NECT

      • IHBD dilation = clue

    • Capsular retraction may reveal intrahepatic tumor

    • Large common duct (extrahepatic) masses may be identified on NECT

  • CECT

    • Solitary, small, well-demarcated tumors are difficult to differentiate from primary hepatocellular carcinoma (HCC)

    • Arterial phase: Peripheral CC seen as intrahepatic mass showing early peripheral rim enhancement and progressive patchy central enhancement

    • Portal phase: Portal vein invasion, ductal wall thickening with minimal enhancement, and portal lymphadenopathy

    • Delayed phase

      • Enhancement with increasing attenuation seen in up to 74% of lesions, usually ↑ CT sensitivity/specificity

      • Persistent tumor enhancement due to fibrous stroma

    • Low reported sensitivity for small hilar lesions (approximately 50%)

    • Regional lymph node spread rarely detected (24-40% of cases)

Nuclear Medicine Findings


    • Primary uses

      • Identification of new lesions

      • Evaluation of metabolic activity and associated malignancy

      • Characterization of response to neoadjuvant therapy

      • Detection of lesions in liver that are not suspected on US or MR in up to 50% of patients

    • Peripheral CC: Intensely hypermetabolic activity, may be ring-shaped

    • Hilar CC: Low activity with focal nodular or linear branching pattern

      • Lower FDG uptake may be related to tumor size or arrangement of fibrous stroma and mucin pool in tumor

      • Can be difficult to discriminate between extrahepatic tumor itself and FDG-accumulating lymph nodes in perihilar region

      • Extrahepatic CC may have low uptake due to loosely connected cell nests and poor detection with PET due to infrequency of evident mass formation

    • PET sensitivity

      • 61-90% for primary CC

      • 85% for nodular CC

      • 18% for infiltrating CC

      • 65-70% for distant metastases

      • Only 13% for regional or hepatoduodenal mets

    • False negatives are seen with mucinous adenocarcinomas (rare)

    • False positives are seen due to foci of inflammation (e.g., intrahepatic stone)

      • Uptake likely to be seen along tract of biliary stents

    • Primary sclerosing cholangitis (PSC)

      • PET can be used to discriminate between PSC with and without CC

      • Not reliable for early diagnosis of CC in patients with PSC

      • Liver in patients with PSC may have ↑ background signal than those of healthy control patients

  • PET/CT

    • Allows better identification of non-FDG avid tumors & carcinomatosis and helps distinguish stent-related uptake from malignant disease

    • Shown to change oncological management in up to 17% of patients

    • No diagnostic advantage over CECT in detection of intrahepatic CC or primary tumor site of extrahepatic CC

    • Generally cost-effective method, avoids unnecessary surgery

  • Hepatobiliary scintigraphy: Focal photopenic lesion

  • Tc-99m sulfur colloid: Focal photopenic lesion

  • Ga-67 scintigraphy: Variable Ga-67 uptake


Hepatocellular Carcinoma (HCC)

Sep 22, 2016 | Posted by in MAGNETIC RESONANCE IMAGING | Comments Off on Cholangiocarcinoma

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