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Review
. 2021 Feb;37(1):3-9.
doi: 10.1159/000513950. Epub 2021 Jan 19.

Diagnostic Approach to Suspected Perihilar Malignancy

Evgeny Solonitsyn  1 , Alexander Dechêne  2
Affiliations
Review

Diagnostic Approach to Suspected Perihilar Malignancy

Evgeny Solonitsyn et al. Visc Med. 2021 Feb.

Abstract

Background: Cholangiocarcinomas are relatively rare tumors most frequently arising from the epithelium of the hilar bile ducts. The diagnosis is often made in advanced (symptomatic) stages, which accounts for the infavorable prognosis with a 5-year survival of less than 10%. Identification of perihilar cholangiocarcinoma (pCCA) is often challenging because there is no single method offering sufficient diagnostic accuracy.

Summary: Most tumors arise in patients without specific risk factors. Clinical symptoms of pCCA are nonspecific and reliable biomarkers are lacking, with carbohydrate antigen 19-9 being the most commonly used tumor marker (but with a low accuracy). Cross-sectional imaging (CT and MRI) is used to identify and map hilar strictures and determine resectability by showing vascular involvement. Endoscopic ultrasound offers additional information on locoregional tumor spread and lymph node involvement. Endoscopic retrograde cholangiography in combination with cholangioscopy gives direct access to and imaging of hilar strictures but it does not always distinguish between pCCA and benign hilar strictures. Tissue acquisition for histological diagnosis is challenging, with frequent sampling errors regardless of the method of biopsy procurement because of the cellular paucity of tumor tissue.

Key messages: In suspected perihilar malignancy, a mosaic of clinical data has to be taken into account. Histological evaluation of (endoscopically harvested) specimens is pivotal to differential diagnosis. Several new techniques to increase diagnostic accuracy are under investigation (biomarkers and genetic testing among others).

Keywords: Biopsy; Cholangiocarcinoma; Cholangioscopy; Endoscopic retrograde cholangiopancraticography; Endosonography.

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Conflict of interest statement

The authors declare that there are no conflict of interests.

Figures

Fig. 1
Fig. 1
ERC: Hilar stricture extending to both liver lobes (pCCA type Klatskin 4).
Fig. 2
Fig. 2
Percutaneous transhepatic cholangiography and drainage (PTCD) (right liver lobe) in a patient with a malignant hilar obstruction.
Fig. 3
Fig. 3
Cholangioscopic appearance of a malignant hilar stricture with visible tumor vessels.
Fig. 4
Fig. 4
Cholangioscopically guided biopsy of a suspicious intraductal lesion.
Fig. 5
Fig. 5
Periportal lymph nodes (during EUS-guided FNA).
Fig. 6
Fig. 6
IDUS of a smooth excentric biliary stricture without distinct features of malignancy.
Fig. 7
Fig. 7
Diagnostic workup in patients with a suspected hilar malignancy.
See this image and copyright information in PMC

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