cir esp.

2015;xx(xx):xxx–xxx

CIRUGÍA ESPAÑOLA

www.elsevier.es/cirugia

Review article

Klatskin Tumor: Diagnosis, Preoperative

Evaluation and Surgical Considerations§

Vı́ctor Molina,* Jaime Sampson, Joana Ferrer, Santiago Sanchez-Cabus, David Calatayud,
Mihai Calin Pavel, Constantino Fondevila, Jose Fuster, Juan Carlos Garcı́a-Valdecasas
Unidad de Cirugı́a Hepática y Trasplante, Institut de Malalties Digestives, Hospital Clı́nic, Universidad de Barcelona, Barcelona, Spain

article info abstract

Article history: Hiliar cholangiocarcinoma is the most common type of cholangiocarcinoma, an represent
Received 7 April 2015 around 10% of all hepatobiliary tumors. It is an aggressive malignancy, resectable in around
Accepted 9 July 2015 47% of the patients at diagnosis. Complete resection is the most effective and only poten-
Available online xxx tially curative therapy, with a survival rate of less than 12 months in unresectable cases.
Axial computerised tomography and magnetic resonance are the most useful image tech-
Keywords: niques to determine the surgical resectability. Clinically, jaundice and pruritus are the most
Klatskin tumor common symptoms at diagnosis; preoperative biliary drainage is recommended using
Cholangiocarcinoma endoscopic retrograde cholangiography or percutaneous transhepatic cholangiography.
Prognostic factors Surgery using extended liver resections with an en bloc resection of the liver with vascular
Biliary malignancies reconstruction is the technique with the highest survival. Complete resection with histo-
Criteria of unresectability logically negative resection margins (R0), nodal involvement and metastases are the most
important prognostic factors.
# 2015 AEC. Published by Elsevier España, S.L.U. All rights reserved.

Tumor de Klatskin: diagnóstico, evaluación preoperatoria

y consideraciones quirúrgicas
resumen

Palabras clave: El colangiocarcinoma hiliar es el colangiocarcinoma más frecuente, representando hasta

Tumor de Klatskin un 10% de todos los tumores hepatobiliares. Es un tumor agresivo con una resecabilidad al
Colangiocarcinoma diagnóstico del 47% y una supervivencia sin cirugı́a inferior a 12 meses. Las pruebas de
Factores pronósticos imagen más utilizadas para valorar estadificación y resecabilidad son la tomografı́a
Neoplasia de vı́a biliar computarizada y la colangiorresonancia magnética. La mayorı́a de los pacientes presentan
Criterios de irresecabilidad prurito e ictericia al diagnóstico, por lo que el drenaje biliar preoperatorio está indicado,
pudiendo realizarse por colangiopancreatografı́a retrógrada endoscópica o colangiografı́a
transparietohepática. En la actualidad, el único tratamiento curativo consiste en la resec-
ción quirúrgica, siendo la resección amplia con resección en bloque y reconstrucción

§
Please cite this article as: Molina V, Sampson J, Ferrer J, Sanchez-Cabus S, Calatayud D, Pavel MC, et al. Tumor de Klatskin: diagnóstico,
evaluación preoperatoria y consideraciones quirúrgicas. Cir Esp. 2015. http://dx.doi.org/10.1016/j.ciresp.2015.07.003
* Corresponding author.
E-mail address: vmolina@clinic.ub.es (V. Molina).

2173-5077/ # 2015 AEC. Published by Elsevier España, S.L.U. All rights reserved.

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2 cir esp. 2015;xx(xx):xxx–xxx

vascular la técnica que ha conseguido una supervivencia mayor a largo plazo. La resección
R0, la afectación ganglionar y las metástasis a distancia siguen siendo los factores pro-
nóstico más importantes.
# 2015 AEC. Publicado por Elsevier España, S.L.U. Todos los derechos reservados.

slightly higher overall survival in the PHC group (20% vs 29%,

Introduction respectively; P=.057), so that it was concluded that they are
comparable in terms of treatment and survival. With the
This neoplasia, which originates in the epithelium of the improved diagnostic tests and histopathological knowledge,
biliary ducts, or cholangiocarcinoma (CC), represents 10% of in the future we may be better able to define whether they are 2
hepatobiliary tumours and 2% of malignant tumours.1,2 CC can distinct entities or if they are clinically and biologically the
be divided into 3 subtypes, depending on their anatomical same.
origin within the biliary duct: intrahepatic or peripheral CC
(ICC), perihilar CC or Klatskin’s tumour (PHC) and distal CC.
PHC, which is the object of this revision, is the most
Histopatological Characteristics
frequent, and it represents around 40%–60% of all CC.2,3 This
tumour is aggressive and silent, with non-specific symptoms 90% of PHC are adenocarcinomas. 10% are adenosquamous or
until advanced stages, leading to late diagnosis and short squamous carcinomas, which in some cases have been
survival without surgery of from 6 to 12 months.4 Surgery, associated with a history of lithiasis, cysts or anomalies of
which is the only available curative option, is only possible in the biliary ducts. According to the appearance of the tumour,
approximately 47% of patients at the moment of diagnosis.5–8 growth type and the biological and clinical behaviour of PHC,
The most important prognostic factors for this tumour are they are classified as16,17:
usually associated with surgical options, and tumour stage,
size, ganglia and vascular involvement, intrahepatic metas- - Tumour or ‘‘mass-forming’’ CC: this is the most common form
tasis and histological type are the most important factors.9–11 of presentation in ICC, although it can also be found in a
large number of PHC. It is characterised by the formation of a
tumour mass with clearly defined margins. It has a major
Anatomical Space fibrotic reaction and central necrosis is also frequent. This
tumour originates in the opening of the biliary duct,
The anatomical space occupied by PHC would be delimited by invading the wall and disseminating by growing three-
the entry to the cystic duct at distal level, and the bifurcation dimensionally, forming a nodular mass that gives rise to
of the right and left hepatic ducts at the proximal level.1,12 obstructive symptoms.16–19
The most widely used classifications include all of the CC that - Infiltrating periductal CC: tumours of this type grow along a
originate in the biliary confluence or its surroundings. Some biliary duct in the form of a concentric longitudinal
groups have suggested that the CC originating in the hepatic thickening through the connective tissue around the duct,
parenchyma sometimes can invade the biliary confluence, causing stenosis or complete obstruction of the affected
with an origin in the anatomical space delimited by the source biliary duct.19 The majority of PHC are of this type, and they
of the rear right portal vein branch and the falciform are difficult to identify using imaging techniques.17
ligament.13–15 These ICC involving the biliary confluence - Intraductal or intraductal papillary CC: this variety is char-
would be treated in the same way as tumours with an acterised by the presence of superficial and intraluminal
extrahepatic origin, and survival is similar to PHC in the same tumours in the biliary duct. They produce mucin and cause
stage. They are usually highly developed tumours with the partial obstruction and dilatation of the ducts.16 This
locoregional vascular and lymph node invasion. Doubts about tumour has a low degree of malignancy and is usually small
the biological behaviour of these tumours have led many in size, although it may spread through the biliary mucus,
groups to exclude them from perihilar tumours. In a study giving rise to multiple tumours (papilomatosis or papillary
published by Ebata et al.13,15 of 250 patients resected for CC carcinomatosis).20
with involvement of the confluence, stage and survival were
analysed according to whether the tumour was intrahepatic The importance of this differentiation lies in the variations
with involvement of the confluence (ICC), or if the tumour in survival depending on the subtype in question. Several
origin was in the extrahepatic biliary duct (PHC). A total of studies have shown that patients with the intraductal
83 patients presented ICC and 167 PHC. When stages were papillary type have a better prognosis than those with the
compared, patients with ICC displayed a higher frequency of scleral-nodular varieties,21 with an average survival of 55 and
vascular and lymph node involvement, with a TNM higher 33 months, respectively,22 while vascular and ganglion
than that of the PHC group, presenting stages III and IV in 59% of involvement are less frequent in the intraductal papillary
cases, in comparison with 38% in the PHC group. Nevertheless, subtype.
if survival is divided according to stages in both groups, there To summarise, the majority of PHC is adenocarcinomas
are no statistically significant differences at 5 years, with with a periductal growth pattern that gives them a poorer

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prognosis, while the variant with intraductal growth is the Of the postoperative classifications, the most widely used is
least frequent, although it has a better prognosis. the TNM classification of the Union for International Cancer
Control (UICC), seventh edition.12 This classification takes
pathological data into account, such as local extension,
Staging Systems vascular involvement, lymph node involvement and metas-
tasis, to establish a classification that includes the extension
Staging systems for PHC may be differentiated into pre- and of the tumour and is prognosis in nature.
postoperative classifications. Of the first, which are used when Recently the ‘‘Japanese Study Group on Perihilar Cholan-
planning surgery, the most important system is the Bismuth- giocarcinoma’’ (JSGPH) published a study which proposed
Corlette classification. This indicates which lobe is preferen- modifying the classification of the ‘‘Union for International
tially affected, and it therefore shows the type of hepatectomy Cancer Control’’. The basic differences are shown in Table 1,
which should be used (Fig. 1).23 This classification was and they chiefly consist of13:
invented in the 1970s and takes neither lymph node
involvement nor metastasis into account, so that it now has 1. Not considering Bismuth IV to be T4. The classification of
less prognostic value. the JSGPH therefore does not take bilateral biliary extension
Another preoperative classification used in the USA is the into account as a poor prognosis if an R0 resection is
one published by Jarnagin et al.10 of the Memorial Sloan achieved.
Kettering Cancer Centre, New York. This classification aims to 2. With respect to the stages, it prioritises lymph node
predict the resectability of tumours, taking 3 local extension involvement as the worst prognosis. They therefore
factors into account. These are biliary extension, vascular consider lymph node involvement to be stage IVa and not
involvement and lobe atrophy. This classification takes IIIb (TNM7).

neither lymph node involvement nor metastasis into account,

so that it has less prognostic value. As resectability differs At a European level an international registry of perihilar
from group to group, while the tendency is towards increa- tumours treated surgically has been created, led by the
singly aggressive treatments, its value will depend on the ‘‘International Cholangiocarcinoma Group for the Staging of
criteria for non-resectability of each centre. Local involvement PHC’’. This group has published a new classification1 which
should now not be a criterion for non-resectability, on takes tumour size into account, together with biliary, venous,
condition that we are able to achieve an R0 with surgical arterial, ganglion and metastatic involvement, preoperatively
resection. as well as postoperatively. Nevertheless, the restrictions of
this classification are that it merely describes tumour
characteristics and the surgery to be performed, without
going on to divide patients into groups or stages according to
these variables. As a result is it not possible to extract
prognostic conclusions and it is also impossible to compare
the results of different groups.
Previous publications show that the preoperative classifi-
cation used the most widely now to decide on the type of
resection is Bismuth-Corlette, while the TNM 7 classification is
used to define the long-term prognosis.

Diagnostic Strategies

The symptoms associated with PHC more frequently are:

jaundice (90%), weight loss and abdominal pain (35%), pruritus
(26%) and acute cholangitis (10%).17 Due to these symptoms
the initial diagnosis is usually made using by abdominal
ultrasound, and this is a good screening test. Other diagnostic
tests for this type of tumour would be:

 Abdominal Doppler Ultrasound: this makes it possible to

evaluate arterial and portal permeability (thrombosis),
which may condition the resectability of the tumour and
therapeutic strategy, although it is not the test of choice for
the evaluation of vascular involvement.
 Abdominal computerised axial tomography: this is useful for
diagnosis of the primary tumour and disease extension,
with 80% sensitivity in the evaluation of biliary extension. It
is the technique of choice for preoperative evaluation of
Fig. 1 – The Bismuth-Corlette23 classification. vascular anatomy, with a sensitivity of 93% and 87% for the

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Table 1 – Comparison of the Basic Differences Between the UICC TNM 7 Classification and the Proposal by the JSGPH.

Tumour classification UICC JSGPH proposal

Tis Carcinoma in situ
T1 Tumour confined to the biliary duct
T2a Surpasses the duct wall and invades
perihilar adipose tissue
T2b Invades the hepatic parenchyma
T3 Unilateral invasion of the hepatic artery/vein
T4 Invasion of the main branch of the HA/PV, Vascular invasion of the main brand
Bismuth IV, or Bismuth III with contralateral of the HA/PV, with bilateral vascular
invasion of the A/V or contralateral invasion

TNM stage
0 Tis N0 M0
I T1 N0 M0
II T2 N0 M0
IIIa T3 N0 M0
IIIb T1-3 N1 M0 T4 N0 M0
Iva T4 N M0 T N1 M0
Ivb T N M1
HA, hepatic artery; JSGPH, Japanese Study Group on Perihilar Cholangiocarcinoma; TNM, tumour lymph node metastasis; UICC, Union for
International Cancer Control; PV, portal vein.
Source: Ebata et al.13

evaluation of arterial and portal involvement, respectively. lesser extent, without specifying their location or char-
It has low sensitivity for the preoperative evaluation of acteristics. The levels in serum of these markers are
lymph node involvement (50%).14,24 It is also useful in strongly influenced by biliary obstruction and jaundice
performing volumetric tests and calculating the hepatic due to their biliary elimination. Different normal values
volume remaining after surgical resection. It is also the most have been proposed, depending on the presence of
economical test for preoperative staging. hepatopathy (300 U/ml) or jaundice (1000 U/ml), while in
 Magnetic resonance and magnetic resonance cholangiography pancreatic cancer sensitivities higher than 70% are
(RM-cholangiography): the best test for the diagnosis of the obtained, with levels of specificity higher than 95%. Its
primary tumour and to evaluate biliary extension. It has a concentration in these patients varies widely and does not
sensitivity of 86%–100%, and it is better than direct correlate with tumour size, although it does correlate with
cholangiography, while it is also a non-invasive test.25 On metastatic involvement. The sensitivity and specificity of
the other hand, it has low sensitivity for the evaluation of this marker can be increased by combining it with CEA,
vascular involvement (73%) and a sensitivity of 80% for above all in Lewis A negative cases (non-producers of CA
invasion of the hepatic parenchyma. 19.9).30
 Direct cholangiography: endoscopic retrograde cholangiopancrea-
tography (ERCP) and transparietal hepatic cholangiography It may be deduced from the above data that we usually
(TPHC): these inform us about the level of biliary obstruction commence study using an ultrasound scan for the diagnosis
and make it possible to take samples from the lesion for of PHC. This takes place in the context of a patient with
cytology, with a sensitivity of 20%. They have now been jaundice, while computerised tomography and MR-cholan-
replaced in diagnosis by MR-cholangiography. They are very giography are the best staging tests and the most recom-
useful for preoperative biliary drainage and in the palliative mendable prior to surgery. MR or computerised tomography
treatment of PHC, with the insertion of preferentially metal- may be used for volumetric testing, depending on the type of
coated stents.26–28 apparatus or computer programmes available in each
 Endoscopic ultrasound: this is useful for the evaluation of hospital. If there is doubt about spread into the lymph
ganglion involvement in the area of the celiac trunk nodes which may contraindicate surgery positron emission
and peripancreatic region, establishing preoperative tomography or fine needle aspiration endoscopy is recom-
staging and making it possible to take fine needle aspiration mended.
biopsies.
 Positron emission tomography: this is useful in the study of
patients with suspicion of metastatic involvement as well as
Non-Resectability Criteria
involvement of adenopathies in the celiac trunk. Its
sensitivity is controversial, and it may vary from 38% to Surgery is the only curative treatment for PHC, and it offers
90%, depending on the series published.14,24,29 the best long-term survival. The criteria adopted for surgical
 Tumour markers: these are of limited usefulness, and CA 19.9 resection have expanded over recent years, from those
is the most commonly used. The majority of studies have described initially by the team of the Memorial Sloan
evaluated these in pancreatic neoplasias and in CC to a Kettering Cancer Center by Burke et al.31 in 1998, until the

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recent introduction of approaches using vascular resection drainage in patients with jaundice increases associated
and extended hepatectomies.3,5,32–34 The criteria for non- morbidity without improving survival, mainly increasing
resectability vary from hospital to hospital, and the most complications involving infections. The European multicen-
widespread are: vascular involvement on one side with tre study published in 2013 by Farges et al.38 retrospectively
contralateral biliary involvement up to the division of second- analysed 366 patients who had been subjected to hepatec-
level radicals, distant hepatic metastases, vascular involve- tomy or extended hepatectomy and biliary resection due to
ment of both hepatic lobes, extrahepatic or peritoneal PHC. They were classified according to whether or not
involvement and adenopathic involvement of the celiac preoperative biliary drainage had been performed. The group
trunk, the upper mesenteric artery or the paraaortic without preoperative drainage (non-PBD) was composed of
region.3,5,8,31 186 patients, and the group with biliary drainage (PBD)
In a multicentre study published by De Jong et al.34 which contained 180 patients. The groups were homogeneous in
analysed 305 patients operated for PHC in 7 different centres terms of age, tumour stage and portal resection. The PBD
in the USA and Europe, and which included patients with group presented more right hepatectomies (56% vs 44%).
portal involvement, in multivariable analysis the only 2 When both groups were compared according to the type of
statistically significant prognostic factors were involvement surgery performed, those patients subjected to right hepa-
of the resection margin and lymph node involvement (P=.02). tectomy showed a higher number of postoperative liver
In the study published by Ebata et al.,15 analysing 1352 failure if they belonged to the non-PBD group, with an
patients operated in 8 Japanese hospitals for PHC with incidence of 16% vs 4% in the PBD group (P=.009). In the
curative intent, multivariable analysis of the statistically multivariable analysis of the factors associated with higher
significant prognostic factors showed them to be: vascular mortality in the right hepatectomy group, having bilirubin
invasion, invasion of the pancreas, lymph node involvement, levels under 3 mg/dL before surgery was a statistically
the presence of metastasis and involvement of the resection significant factor. However, if both groups of left hepatectomy
margin. Lymph node involvement and metastasis were the patients were compared, the PBD group presented a higher
factors which led to poorer survival at 5 years in comparison number of postoperative sepsis, with an incidence of 6%,
with the others (10%, 20% and 63%, respectively). These compared to 0% in the non-PBD group (P=.014). This study
results support the use of surgery for these tumours, if in spite shows that although it is true that biliary drainage increases
of local extension it is possible to perform a R0 resection, the incidence of postoperative sepsis, biliary drainage should
given that it is possible to increase survival to 5 years be performed in those patients who are going to be subjected
regardless of local extension. to right hepatectomy to reduce the morbimortality associated
Due to all of the above considerations it is recommended with postoperative liver failure, and other studies support
that each case be evaluated individually, and that surgery be this theory.39 Given that in the treatment of PHC the only
used if an R0 resection can be achieved in the absence of studies which have shown greater survival are those which
distant metastasis or peritoneal involvement. Bilateral biliary support extensive resections to achieve R0 resection, biliary
and local vascular involvement should therefore not be non- drainage is recommended when surgery is indicated, and it
resectability criteria if it is possible to operate while preserving may eventually require extensive hepatectomy of more than
more than 30% of liver volume and achieving an oncological 50% of hepatic volume or trisegmentectomy, or if there is
resection. cholangitis. There is controversy about the cut-off point in
bilirubin levels to indicate drainage, and >10 mg/dL is one of
the most widely used.5,37 It is recommended that biliary
Therapeutic Strategies drainage by CTPH be performed, with emplacement of
external drainage, which avoids manipulation of the tumour.
Biliary Drainage This has a lower incidence of infections than CPRE and makes
it possible, in those patients with unilateral drainage who do
Given that the majority of PHC patients debut with jaundice, not normalise their bilirubin levels, to use bilateral biliary
one of the most important dilemmas regards the utility drainage.35,40
of preoperative biliary drainage. However, this is not free of Tumour dissemination at the puncture site has been
complications, and those associated with ERCP with the described in up to 5%–10% of cases in which CTPH was
insertion of a stent are: pancreatitis, duodenal perforation, used,14,24 although these studies do not specify when external
duodenal migration, catheter obstruction and, most impor- or internal–external drainage was used, and they do not take
tantly, cholangitis. Drainage by CTPH presents a lower into account the time passed until surgery. Some authors
frequency of preoperative cholangitis, but it is associated recommend the use of endoscopically positioned nasobiliary
with haemorrhage, catheter migration, up to 5% tumour drainage to prevent dissemination at the point of puncture,
dissemination within the trajectory of the catheter and with a lower incidence of cholangitis and obstruction of the
discomfort and pain in the entry zone. Overall, according to stent than is the case with CPRE.41 However, the same studies
the published studies, ERCP has an associated morbidity of admit the difficulty of preoperative bilateral biliary drainage
60%, and the corresponding figure for CTPH is 31%.14,35 using this system, and this hinders normalisation of bilirubin
Due to the above reasons, the utility of preoperative biliary levels prior to surgery in patients in which unilateral drainage
drainage in hepatobiliopancreatic surgery has been called has failed.42
into question.36,37 These studies, which include all types of To reduce the morbidity associated with infections
hepatobiliary surgery, have shown that preoperative biliary following preoperative biliary drainage, it is suggested that

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the bile be systematically cultured following drainage and hepatic metastasis, thereby preventing unnecessary laparo-
during surgery. Several studies have shown that 78%–94% of tomies.50–52 Non-invasive imaging techniques are recom-
these cultures are positive for drained patients, as opposed to mended in advanced stages (T2/3/4) that present possible
20%–30% for undrained patients,43–45 and enterococcus is the advanced peritoneal or lymph node involvement, and which
most commonly isolated organism. These groups defend cannot be punctured using echo-endoscopy before creating
the use of prophylactic antibiotics, which although they the preoperative biliary drainage. We always perform
increase the antibiotic resistance of the species isolated, in staging laparoscopy using intraoperative ultrasound scan
published studies this is shown to achieve a postoperative to improve sensitivity to locorregional and lymph node
infection morbidity similar to that of undrained groups. involvement.53
The antibiotic selected will depend on the cultures and the
antibiogram, although the majority of the groups used at Surgical Treatment
least a third generation cephalosporin or fluoroquinolones +
metronidazole, when no culture was available or when it was Surgery is still the only curative treatment for PHC,3,14 and it
negative.43,44,46 achieves a 20%–40% survival rate at 5 years (Table 2).10,11
Palliative biliary drainage is used in patients who cannot Surgical resection must always be performed if it is possible to
undergo resection. Drainage can be by ERCP, leaving a achieve a R0 resection. The main factors which affect survival
coated metal stent, or by CTPH in those cases where it is following surgery are involvement of the resection margins,
impossible to achieve correct drainage of both biliary ducts either microscopically (R1) or macroscopically (R2) together
using ERCP. with lymph node involvement (N1 and N2).14,17,44,54 Several
Thus definitively, preoperative biliary drainage should be studies have shown that survival increases if the resection is
used in all patients with bilirubin above or equal to 10 mg/dL broadened to create negative margins.3,5,33,45 The proposed
and in those where hepatic resections will be greater than 50% techniques include extended hepatectomies with resection of
of hepatic volume. The type of approach depends on the the caudate segment, biliary resection, hilar lymphadectomy
hospital, although CTPH with external drainage is recom- up to the celiac trunk and reconstruction with hepatojeju-
mendable to avoid manipulation of the tumour and the lower nostomy.
incidence of cholangitis. Some authors recommend that The ‘‘no-touch technique’’ first described by Neuhaus
drained patients receive prophylactic antibiotics suitable for et al.32 involves the block resection of the hepatic hilum
their biliary cultures. without manipulation of the tumour, increasing patient
survival to 5 years in more than 15% of cases.3,33,34,55 This
Preoperative Portal Embolisation technique basically consists of the resection of the portal
vein and right hepatic artery when they are close to the
Preoperative portal embolisation was described in the 1980s, tumour, thereby preventing its dissection if this involves
initially by Makuuchi et al.47 and then by Kinoshita et al.48 The manipulation of the tumour, together with complete biliary
aim of portal embolisation is to increase the remaining hepatic resection with broad lymphadenectomy up to the root of the
volume in those cases in which it is considered insufficient in celiac trunk and reconstruction with hepaticojejunostomy. It
the preoperative volumetry, reducing the probability of will be preferable to select the right hepatic lobe (the
postoperative liver failure. In a metaanalysis published essential ‘‘non-touch technique’’, according to Neuhaus),33
recently by Higuchi and Yamamoto49 that included on the condition that tumour extension makes it possible to
836 patients with PHC with preoperative portal embolisation, include the right hepatic artery, which is the one closest to
morbidity was 1% and mortality 0.09%. Tumour progression the tumour. This type of radical surgery leads to an
during the procedure that prevented surgery amounted to
19.4%. These results support the idea that its use in PHC is
justified in those patients with a remaining preoperative
hepatic volume of less than 30%.
Table 2 – Survival Following Resection of the PHC
According to the Series Published.
Staging Laparoscopy
Author Year Cases Survival at
5 years (%)
With the advances in the sensitivity and specificity of non-
invasive imaging tests over recent years, staging laparoscopy DeOliveira et al. 2007 35 10
Lladó et al. 2008 62 43
is falling into disuse. The most important criteria for non-
Figueras et al. 2009 19 63a
resectability are lymph node involvement, biliary extension
Unno et al. 2010 125 35
and vascular invasion. These are difficult to evaluate during Young et al. 2010 51 29
laparoscopy, the precision and efficacy of which have been Igami et al. 2010 298 42
falling for years. This is shown by the recent revision by Van Gulik et al. 2011 38 33
Rotellar and Pardo,50 in which precision and efficacy stood at De Jong et al. 2012 305 20.2
41% and 72%, respectively, in 2002, and at 14% and 32% in Neuhaus et al. 2012 100 43
Regimbeau et al. 2014 331 53
studies published in 2011. This fall is chiefly due to
improvement in non-invasive tests such as MR-cholangio- Survival following hepatic resection due to perihilar cholangio-
carcinoma.
graphy. Its efficacy increases if patients are selected who are a
Only 2 years follow-up.
at high risk of presenting peritoneal dissemination or

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Fig. 3 – Right hepatectomy with reconstruction of the portal

vein.

Conclusions

Currently, the only curative treatment consists of surgical

resection. Radical en-bloc resection and vascular reconstruc-
Fig. 2 – Left hepatectomy for Klatskin’s tumour, with tion is the technique which has achieved the highest rate of
resection of the portal vein: (A) right hepatic artery of the survival in the long term. To reduce postoperative morbi-
upper mesenterium; (B) portal vein. mortality, it is recommendable to perform biliary drainage to
prevent liver failure, with antibiotic prophylaxis after drainage
and portal embolisation when the future remaining hepatic
volume will be less than 30%. R0 resection, lymph node
acceptable rate of postoperative morbidity, of 50%-70% involvement and distant metastasis are still the most
depending on the series in question, with a mortality of important prognostic factors.
10%–20%.3,5 PHC patient survival at 5 years stands at 20% to
40%, while studies published that include portal resection
and series using the ‘‘no-touch technique’’ achieve 58% at
Conflict of Interests
5 years (Figs. 2 and 3).3,5,33
It is also important to underline recent studies which This revision has not been presented or published partially or
show an increase in the survival of patients with preope- wholly in any journal or congress.
rative vascular involvement following portal resection. They
even achieve rates of survival that are equal to or higher than
references
those for patients without preoperative vascular involve-
ment and who were therefore not subjected to portal
resection.33,56,57 In these studies, the incidence of hepatic
1. Deoliveira M, Schulick R, Nimura Y, Rosen C, Gores G,
and vascular complications are similar to those in the group
Neuhaus P, et al. New staging system and a registry for
without portal resection, except for those patients subjected perihilar cholangiocarcinoma. Hepatology. 2011;53:1363–71.
to arterial resection, as these present a higher rate of 2. Nakeeb A, Pitt HA, Sohn TA, Coleman J, Abrams RA,
morbimortality than the others. Due to all of these Piantadosi S, et al. Cholangiocarcinoma. A spectrum of
considerations, surgery is recommended for those patients intrahepatic, perihilar, and distal tumors. Ann Surg.
with unilateral portal involvement or involvement of the 1996;224:463–73.
3. Van Gulik TM, Ruys A, Busch ORC, Rauws EJ, Gouma DJ.
confluence in preoperative tests, performing an en-bloc
Extent of liver resection for hilar cholangiocarcinoma
resection and vascular reconstruction. Arterial resection and
(Klatskin tumor): how much is enough? Dig Surg.
reconstruction are not recommended unless the artery is 2011;28:141–7.
clearly affected, as this is associated with poorer postope- 4. Habermehl D, Lindel K, Rieken S, Haase K, Goeppert B,
rative outcomes, so that in such cases right hepatectomy is Büchler MW, et al. Chemoradiation in patients with
preferable. unresectable extrahepatic and hilar cholangiocarcinoma or
To summarise, the surgical principles to be followed will at high risk for disease recurrence after resection: analysis
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