Practical review for diagnosis and clinical management of perihilar cholangiocarcinoma

Table 1 Patients that should undergo to screening programs and the techniques that should be applied

Predisposing factors	Diagnostic technique	Worrisome features
Intrahepatic lithiasis and recurrent pyogenic cholangitis	MR	Stenosis progression, distal bile duct dilatation, intraductal polypoid mass > 1 cm.
PSC	MR + ERCP	Irregular bile duct stenosis, bilateral bile duct dilatation, ipsilateral lobar atrophy. ERCP bile duct sampling can simplify differential diagnosis.
Intrahepatic fluke	US/MR	Central intrahepatic and main bile duct dilation with stenosis identificationa.

MR: Magnetic resonance; PSC: Primary sclerosing cholangitis; ERCP: Endoscopic retrograde pacreatoduodenoscopy; US: Ultrasound.

^a: Ultrasound, computed tomography and magnetic resonance findings for intrahepatic fluke are diffuse and uniform dilatation of peripheral intrahepatic bile ducts with no dilatation of central intrahepatic and main bile ducts, without focal obstruction lesions, with increased echogenity of bile ducts and non-shadowing echogenic foci within bile ducts (eggs or flukes).

Table 2 Main advantages of two the two techniques available to obtain bile duct drainage

ERCP	PTC
Internal stent: Less patient discomfort[5]	External drainage: Increased patient discomfort[5]
Reduced risk of seeding[73]	Higher expertise needed[108]
Higher rate of bacterial contamination/cholangitis[76]	Higher rate of hemorrhage[76]
“One shot” microbiological examination	Never cross the malignant bile duct stenosis[5]
Removed during surgery	Repeated cholangiography and microbiological samples
	Useful during and after surgery

ERCP: Endoscopic retrograde pacreatoduodenoscopy; PTC: Percutaneous transhepatic colangiography.

Table 3 Criteria that can be used to identify non-resectable patients

Absolute criteria	Relative criteria
	Criteria	Suggestions
Presence of distant metastasis (especially liver, lung, peritoneum)	Longitudinal and lateral dissemination	Consider adequate staging (avoid R1-2)
Extra-regional lymphnode involvement (para-aortic and extraperitoneal)
Bilateral intrahepatic involvement of biliary tree that exclude bilio-enteric anastomosis	Portal infiltration < 2 cm	Portal vein resection needed
Infiltration or occlusion of the main portal trunk proximal to bifurcation
Right lobe atrophy associated to contralateral portal vein infiltration or portal occlusion > 2 cm	Low remant liver	Consider liver hypertrophy techniques
Right lobe atrophy associated to contralateral tumor extension more than to 2 cm from hepatic hilum
Contralateral invasion of hepatic artery	Type IV pCCC	High expertise; consider en-bloc resection
Unilobar secondary bile ducts invasion associated to contralateral infiltration or collusion of portal vein

pCCC: Perihilar cholangiocarncioma.

Table 4 Articles reporting resection of type IV perihilar cholangiocarncioma according di bismuth

Author	Publication year	Resection rate (%)	Resected cases (n)	Vascular resection (n)	Vascular reconstruction (n)	Vascular invasion at histological evaluation (n)	Complications (%)	N+ (%)	R0 (%)	Patient survival 1-3-5 yr (%)
Hu HJ	2018	NA	69	52	14	63	39	57	86	76-44-22
Li B	2017	NA	142	42	NA	NA	NA	37	75	35-12-3
Ebata T	2018	50	216	131	NA	136 PV + 53 HA	19	20	72	68-34-22
Ji GW	2017	NA	25	4	4	13	13	76	95	NA
Hoffman K	2015	NA	31 (+29 tipo II e III)	3112	211	19	52	36	60	84-38-181
Han IW	2014	21	33	6	NA	12 PV + 13 HA	NA	36	54	NA-28-NA
Cheng QB	2012	61	101 (+75 tipo III)	NA	NA	NA	25	40	76	89-38-133

We selected only the series were data on type IV resection can be extract.

¹Added type II and III cases.

²All cases with presumed vascular infiltration had vascular resection, while only 19 cases had histological proven vascular infiltration.

³Added type III cases. PV: Portal vein; HA: Hepatic artery. NA: Not applicable.