Intrahepatic cholangiocarcinoma (iCCA) is the second most common malignant primary liver cancer after hepatocellular carcinoma (HCC). Although most cholangiocarcinomas (CCAs) occur incidentally, iCCA may develop on top of underlying chronic liver disease, and its incidence is increasing with the prevalence of obesity and metabolic diseases. Hepatic hilar cholangiocarcinoma (pCCA) may present after a history of chronic inflammatory disease of the biliary tract. For patients at risk of biliary obstruction, malnutrition, and sepsis, initial treatment of CCA is often complicated. In addition, in the age of personalized medicine, it is critical to ensure that patients have access to continuously improving medical, surgical, and radiation treatments.
Recently, the France Association for the Study of the Liver (AFEF) has developed guidelines for the management of intrahepatic and hilar cholangiocarcinoma, with the aim of proposing the best management plan for all stages of the disease to improve patient outcomes. Yimai Tongte has compiled it for the reference of clinicians.
Risk factors and screening
When bile duct cancer is suspected, the following risk factors warrant systematic screening: cystic and calculous lesions of the biliary tract, primary sclerosing cholangitis, cirrhosis, hepatitis B/C infection, alcohol consumption, diabetes mellitus, and smoking.
Screening for cholangiocarcinoma is not recommended in patients with low phospholipid-associated cholelithiasis (LPAC) syndrome in the absence of large stones. Screening for cholangiocarcinoma is recommended for patients with Caroli's disease. An MRI once a year may be done as a screening option.
The association between cholangiocarcinoma and biliary hamartomas is controversial and rare. Screening for cholangiocarcinoma is not recommended for patients with biliary tract hamartoma.
Screening for cholangiocarcinoma is recommended in patients with primary sclerosing cholangitis (PSC). If contrast contrast is not used for initial MRI, contrast contrast MRI is recommended within 6 months of diagnosis of PSC.
When bile duct carcinoma is suspected, a multidisciplinary team meeting will recommend endoscopy of the biliary tract for tissue sampling.
CA19-9 testing to screen for cholangiocarcinoma is not recommended in patients with primary sclerosing cholangitis.
Diagnosis and pre-treatment testing
1. Diagnosis of iCCA
Multi-period CT of the chest, abdomen, and pelvis is recommended.
In the absence of extrahepatic metastases, MRI of the liver (with MRCP for biliary tract disease) is recommended.
Needle biopsy of liver tumors is recommended to confirm the diagnosis and differential diagnosis.
Systematic FDG-PET staging is not recommended.
After biopsy, if there is still doubt about liver metastases, immunohistochemistry-guided evaluation is recommended to identify extrahepatic primary tumors (digestive endoscopy, FDG-PET).
2. Differential diagnosis of iCCA
When malignant lesions in the liver without cirrhosis are suspected, biopsy is recommended as a guide to supplemental testing.
建议优先考虑以下免疫组织化学组以区分iCCA与肝转移：女性中的CK7、CK20、CDX2/SATB2、TTF1和GATA3。
Secondary use of anti-CRP antibodies is recommended to distinguish CCA from liver metastases.
3. Diagnosis of pCCA
Figure 1 shows the diagnostic and pre-treatment testing strategies for pCCA
Figure 1 Diagnosis and pre-treatment testing strategies for pCCA
treat
Figure 2 shows the overall treatment strategy for iCCA
Fig. 2 The overall treatment strategy for iCCA
Figure 3 shows the systematic treatment strategy for advanced CCA
Fig.3 Systematic treatment strategy for advanced CCA
Overall, cholangiocarcinoma is a rare and heterogeneous cancer that originates in the bile ducts. Its multidisciplinary approach requires a combination of radiology, anatomic pathology, endoscopy, surgery, oncology, molecular and supportive care expertise.
Bibliography:
Neuzillet C, Decraecker M, Larrue H,et al. Management of intrahepatic and perihilar cholangiocarcinomas: Guidelines of the French Association for the Study of the Liver (AFEF). Liver Int. 2024 Jul 5. doi: 10.1111/liv.15948. Epub ahead of print. PMID: 38967424.
Yimaitong is a professional online doctor platform, and the mission of the platform is to "sense the pulse of the world's medicine and help China's clinical decision-making". Yimaitong has a series of products such as "Clinical Guidelines", "Medication Reference", "Medical Literature King", "Yizhiyuan", "eYantong" and "ePulse", which fully meet the needs of medical workers in clinical decision-making, obtaining new knowledge and improving scientific research efficiency.