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Hewitt et al. Hepatoma Res 2021;7:75 https://dx.doi.org/10.20517/2394-5079.2021.83 Page 7 of 19

are encouraged to achieve R0 resection [87,102] . In an earlier study examining 80 patients with PCCA, extended
hepatic resection, including right trisectionectomy and portal vein resections, resulted in higher rates of R0
[102]
resection and subsequent longer survival . In a recent retrospective study, 216 patients with Bismuth type
IV PCCA underwent resection, of which 112 patients underwent a left hepatic trisectionectomy, and 131
patients had a combined vascular resection . Over 40% of patients experienced a Clavien-Dindo grade III
[87]
or higher complication and 1.9% surgical mortality within 90 days. An R0 resection was achieved among
72% of patients. The high R0 resection rate in this challenging patient population corresponded to a
significantly improved 5-year survival rate compared with patients who had unresected tumors (32.8% vs.
1.5%; P < 0.001). As recently as the AJCC 7th edition, type IV PCCAs were classified as stage IV disease and
deemed unresectable due to the poor OS . The feasibility of excellent oncologic outcomes with low
[103]
mortality despite higher morbidity in a surgically complex patient population has been demonstrated. For
example, portal vein resection can be routinely performed when there is suspicion of tumor invasion.
However, arterial resection and reconstruction should be limited to highly selected patients as arterial
resection can result in higher morbidity and mortality compared with portal vein resection . For patients
[88]
with Bismuth type I or II, limited bile duct resection has been attempted; however, en bloc liver resection
typically has provided significantly better 5-year survival (50%) compared with extrahepatic bile duct
[104]
resection alone (30%) and is the preferred management approach .

Lymphadenectomy
The presence of nodal metastases adversely impacts OS in patients with CCA. In addition, when nodal
disease is present, prognostic factors including vascular invasion and multiple tumors no longer impact
[105]
survival, suggesting that nodal spread is one of the most important prognostic indicators in CCA .
[10]
Lymphadenectomy provides prognostic and staging information but offers little therapeutic benefit .
Approximately half of surgeons perform a lymphadenectomy with the utilization of lymph node evaluation
rising proportionally to tumor size . When performed routinely for ICCA and PCCA, lymph node
[106]
metastases appear in 40%-50% of patients, with the incidence increasing proportionally with T stage [107,108] .
Standard dissection for patients with ICCA or PCCA involves a regional lymphadenectomy of the porta
hepatitis . A recent recommendation proposed that lymphadenectomy for ICCA and PCCA should
[47]
include both station 12 (hepatoduodenal ligament) and 8 (common hepatic artery) lymph nodes.
[109]
Lymphadenectomy of these stations covered 82% of metastatic cases regardless of tumor location . While
there is no universal consensus on the minimum number of lymph nodes needed for accurate staging, the
[110]
8th edition of the AJCC staging system recommends recovery of at least 6 lymph nodes . Gross lymph
node metastases to the porta hepatis portend a poor prognosis, and surgical resection should only be
pursued in highly selected patients.

Management of distal cholangiocarcinoma
DCCA can also present later in the disease process, and only a minority of patients are resectable at the time
of diagnosis . Prior to resection, a thorough evaluation of the anticipated proximal biliary margin should
[111]
be performed to confirm the absence of disease. The recommended surgical approach for DCCA is
pancreaticoduodenectomy with lymphadenectomy and typical pancreaticojejunostomy,
hepaticojejunostomy, and gastrojejunostomy or duojejunostomy reconstruction. Patients with R0 resection
have a median survival of 25 months and approximately 30% 5-year survival [112,113] . The morbidity profile
compares favorably to more proximal CCAs with lower mortality rates . In situations with diffuse
[114]
involvement of the biliary tract where the CCA extends to include the distal and perihilar segments, a
hepatectomy can be added to a pancreaticoduodenectomy to achieve negative margins with acceptable
morbidity and mortality [115,116] . These combined cases should be performed at high-volume centers with
experienced multidisciplinary care teams for highly selected patients to achieve the best outcomes.