CME-Certified Training

The surgeon
February 2014, Volume 85, Issue 2, pp 155-168
First line: 26 January 2014
Central cholangiocarcinoma (Klatskin tumor)

GA Stavrou

Summary
The perihilar cholangiocarcinoma (Klatskin tumor) is a rare tumor, starting
from the extrahepatic bile duct fork. Given the close relationship of the
anatomical biliary fork to liver parenchyma, and hepatic arteries
Pfortadergabel the treatment of these patients represents a major
challenge. With an incidence 2-4 illnesses / 100,000 inhabitants / year,
treatment is only useful to centers that have the necessary
experience.Histologically usually results from a moderately differentiated
adenocarcinoma that can grow diffusely infiltrating along the bile ducts and
Perineuralscheide proximally, but distally. As the only curative option, the
radical surgical resection of bile ducts, bile duct fork en bloc has prevailed
with liver resection and also Gefresektion, yet achieving a proximal and
lateral safety distance from the tumor is technically problematic.
Keywords
Klatskin cholangiocarcinoma hepatectomy imaging operation strategy
Abbreviations
A. / Aa.
Artery / arteries
ALPPS
Associated liver and portal vein ligation partition for staged
hepatectomy
CA 19-9
Carboanyhdrat antigen 19-9
CCC
cholangiocellular carcinomas
CEA
carcinoembryonic antigen
CT
Computed Tomography
ERCP
endoscopic retrograde cholangiopancreatography
IORT
Intraoperative radiotherapy
MRCP
Magnetresonanzcholangiopankreatikographie
MRI

Magnetic Resonance Imaging

PET
Positron Emission Tomography
PSC
primary sclerosing cholangitis
PTCD
percutaneous transhepatic biliary drainage
PVE
, portal venous embolization
V. / Vv.
Vena / Venae
Perihilar cholangiocarcinoma (Klatskin tumor)
Abstract
Perihilar cholangiocarcinoma or Klatskin tumors are a rare entity Arising from
the extrahepatic bile duct bifurcation. Considering the close anatomical
relationship of the bile duct bifurcation with the portal vein bifurcation and
hepatic arteries, surgical treatment is demanding. With an incidence of only
2-4 cases / 100,000 population / year patients Should be Referred to a
specialized center. The tumors are poorly differentiated adenocarcinomas
Usually diffusely growing Along the duct so the perineural sheath and. Only
radical surgery offers a curative option and currently surgical strategy
Usually Consists of en bloc resection of the bile duct, liver resection and
portal vein extended resection. Proximal and lateral safety margin R0
resections are technically very demanding procedures Because of the local
anatomy.
Keywords
Klatskin tumor cholangiocarcinoma Liver resection Imaging Surgical Strategy
Learning Objectives
After reading this article ...

You know the etiology and staging of Klatskin tumors,

Do you know how diagnostic measures for this type of tumor can be
used wisely,

are you the criteria of resectability known

Know the potential surgical resection strategies.

Etiology and Staging
The central cholangiocarcinoma, also perihilres cholangiocarcinoma or after
his Erstbeschreiber Gerald Klatskin [1 called] Klatskin tumor, belongs to the
family of cholangiocellular carcinomas (CCC; [2]). The CCCs arising from
epithelial cells of the intra- or extrahepatic bile ducts. These are basically

divided into intra- and extrahepatic CCCs. The intrahepatic CCC arise from
the small intrahepatic bile ducts or the large bile ducts of the right and left
biliary system proximal to the bifurcation, beginning with the region of the
biliary extrahepatic fork the CCCs. In principle are bile duct tumors that
infiltrate the central bile duct fork, today irrespective of their origin (intra- or
extrahepatic) as perihilres cholangiocarcinoma referred. 50% of all CCCs are
perihilar, 40% distal extrahepatic and intrahepatic tumors only 10% [3]. The
CCCs are beyond the perihilar area according to their anatomical infestation
pattern after the Bismuth-Corlette classification (Fig. 1divided) [4]. After this
classification, vascular or Leberparenchyminfiltrationen will not be
considered, so that they can be used is limited for the assessment of
prognosis. The new TNM classification appears under these aspects more
suitable (Tab. 1).
The CCCs arising from epithelial cells of the intra- or extrahepatic bile ducts

Fig. 1
Classification of central bile duct tumors after Bismuth-Corlette. (Drawing:
Prof. Giuliana Brogi, Siena, Italy, by kind permission.)
Tab. 1
TNM classification of malignant tumors of the International Union Against
Cancer 2010. (Mod. For [42])
T - primary tumor
TX

Primary tumor can not be assessed

T0

No evidence of primary tumor

Tis

Carcinoma in situ

T1

Tumor on the bile duct with limited extension into the muscularis
propria or the fibromuscular layer

T2
a

Tumor invades beyond the bile duct into the adjacent soft tissue

T2
b

Tumor invades adjacent liver parenchyma, the

T3

Tumor invades unilateral branches of the portal vein or hepatic

artery

T4

Tumor invades the main branch of the portal vein or bilateral

branches; or the common hepatic artery or branches 2nd order
bilaterally; or unilateral branches of 2nd order of the bile duct with
infiltration of contralateral branches of the portal vein or hepatic
artery

N - Regional Lymph Nodes

NX

Regional lymph nodes can not be assessed

N0

No regional lymph node metastasis

N1

, Portae Regional lymph nodes metastases in lymph nodes of the

cystic duct, the common bile duct along the hepatic artery and the
vena

M - distant metastases
M
X

Presence of distant metastases can not be assessed

M
0

No distant metastasis

M
1

Distant metastases

The majority of the CCCs (90%) are adenocarcinomas, squamous tumors are
usually the remaining. The adenocarcinomas are further divided into nodular,
sclerosing and papillary carcinoma, the papillary highest resection and cure
rates have [5]. CCCs are rare, its incidence is 1 to 2 cases per 100,000
population.Interestingly, the incidence of extrahepatic CCC appears to be
declining in recent years, however, the intrahepatic CCCs likely to
rise [6]. The spread of cancers can be made longitudinally along the bile
ducts, but also vertically. The growth pattern is diffuse mostly, but there was
also a discontinuous growth described (so-called. "Skip lesions"). With the
newer histopathological techniques can be next to a lymph vessel invasion
also often a perineural growth along the bile ducts in the direction
hepatofugaler observe what the R0 resection significantly more
difficult [7]. It also comes with already small tumors in about 50% of cases of
infiltration of the liver parenchyma or to a vascular infiltration.
The majority of the CCCs (90%) are adenocarcinomas

The spread of cancer can be done longitudinally along the bile ducts, but
also vertically
As the cause of the emergence of a CCC multiple risk factors are
considered. These include recurrent cholangitis (cholelithiasis, primary
sclerosing cholangitis [PSC]), bile duct cysts, chronic hepatitis, but also the
action of chemicals (dioxins, nitrosamines etc.) or drugs (isoniazid,
methyldopa etc.), wherein the primary PSC and biliary cysts which common
risk factors present [8]. In Asia, also parasitic infections in carcinogenesis
play a role. It is estimated that it will take 15 years to develop a CCCs.
The primary PSC and biliary cysts are the most common risk factors
Diagnostic
Patients with central CCCs are usually symptomatic, when the tumor
occludes the bile ducts. The most common clinical signs are jaundice,
pruritus (66%), pain (30-50%), weight loss (30-50%) and fever (20%).Patients
observed due to biliary obstruction pale stools and dark urine. The sequence
of the performed diagnosis should be discussed in an interdisciplinary team
to achieve the best possible success for the patient.
The most common clinical signs are jaundice, pruritus, pain, weight loss and
fever
A histological or cytological securing the malignant process is only possible
in a few cases, due to the difficult visualization of the lesion. The tumor
marker CEA (carcinoembryonic antigen) and CA 19-9 (Carboanyhdrat antigen
19-9) are difficult to interpret at low values, because they are also increased
in cholestasis and cholangitis benigen and therefore can not provide a
decisive contribution to the diagnosis.When clinically reasonable suspicion of
a tumor beyond the perihilar area, the operation is also indicated without
histological confirmation.
When clinically reasonable suspicion that surgery is indicated even without
prior histological confirmation
Imaging
In jaundiced patients the ultrasound finds frequent application. Typical
findings are dilated intrahepatic bile ducts and a lack of connection between
the left and right bile duct. The ultrasound is very well suited for screening
may present vascular infiltration. The endoscopic ultrasound does not matter
much in the diagnosis of proximal bile duct tumors generally. In the first
place the diagnosis before any manipulation of the bile duct MRI with MRCP
(Magnetresonanzcholangiopankreatikographie) to assess the biliary
conditions without the influence periinterventioneller inflammatory
responses (imaging of choice) is recommended. Here already first
conclusions of an operational strategy can be drawn. The method requires no
direct contrast agent in the biliary system (Fig. 2). The MRCP also provides 3-

D representations of the bile duct system.The MRCP is more appropriate to

describe the extent of the tumor, as ERCP (Endoscopic retrograde
cholangiopancreatography), but tends to "Under staging" [9]. The MRCP
should be in front of a relief of cholestasis (ERCP / PTCD [percutaneous
transhepatic biliary drainage]) carried out due to a consecutive collapse of
the duct system.
In the first place the diagnosis before any manipulation of the bile duct MRI
with MRCP is recommended

Fig. 2
Magnetresonanzcholangiopankreatikographie: 78-year-old patient with a
Klatskin tumor type II, the stenosis begins just above the mouth of the cystic
duct and ends in the hepatic
Computed tomography (CT) of the chest and abdomen allows a sufficient
staging with respect to tumor extension and distant metastases, a positron
emission tomography (PET) -CT was evaluated by several research groups,
but brings no significant information gain for most
patients [10]. The triphasic high-resolution CT allows a good statement
regarding the individual anatomy, possible hilar vascular infiltration in portal
venous and arterial system and can also be a virtual surgical planning serve
the vascularization of the liver with regard to the Resektionsstrategie and a
relatively accurate volumetrics of residual liver segments is
possible [11]. The venous drainage can be properly assessed, particularly
with regard to the Resektionsstrategie (right vs. left). If a PTCD catheter in
situ can be moved into the CT also a fourth phase, with a 3-D segmentation
of the biliary tract is possible.
The thoracic and abdominal CT is adequate for a staging in terms of tumor
extent and distant metastasis
PTCD / ERCP
For the relief of biliary ERCP associated with stent placement and the PTCD
available. Most patients will be assigned only after a successful ERCP away at
a center, so that the diagnosis is further complicated by the manipulation of
the bile ducts. Advantage of ERCP is the possibility of sampling for
histological confirmation - this, however, is rarely successful. Disadvantages
are the bacterial contamination to the intrahepatic bile ducts with increased
infectious complications and the potential tumor cell displacement during the
necessary intraoperative stent removal. A tumor cell displacement within the
meaning of implantations or increased incidence of liver metastases was,
however, also postulated for PTCD [12]. The PTCD seems ERCP in assessing
proximal tumor extension superior, but also provides only about half the
cases, a precise analysis.

Advantage of ERCP is the possibility of sampling for histological confirmation

Important for the relief of biliary drainage is a collusion between endoscopist
and surgeon, as it should be decided jointly on the necessity and form of
drainage - is further drainage of future residual liver after surgery usually
sufficient [13]. From the surgical point of view PTCD ERCP (is preferable
because a more accurate and reproducible cholangiography enables easy
and contamination of the biliary tract is minimized 3 fig.).Also possible to
dispense with a drainage after percutaneous cholangiography, when only a
slight cholestasis is present [14]. However, in the area of the left lobe of the
percutaneous access is linked much more difficult and higher
morbidity [1, 15]. With the increasing technological development and
dissemination of ERCP however PTCD is increasingly becoming the exception
process. Even Japanese groups who propagated this always have, and in
particular the new possibilities of Fluoreszenzcholangiographieresponded and
their preoperative approach towards ERCP changes [2, 16, 17].
After percutaneous cholangiography can be dispensed with a drainage if only
a mild cholestasis present

Fig. 3
Percutaneous transhepatic biliary drainage (PTCD) in a patient with a tumor
type Klatskin IIIa. In of failed endoscopic retrograde cholangiography a PTCD
was introduced on both sides to relieve the biliary tract. The right drainage is
advanced into the duodenum. In the Cholangiography the stenosis can be
seen, which extends into the common hepatic duct
Dexter. A cholangiography right biliary system and b left biliary system
Preoperative drainage
The question of whether a drainage of the bile duct system is necessary, is
controversial [3, 14, 18, 19, 20].Stents should be avoided in
principle. Cholestasis of the liver, however, increases the dysfunction, which
is responsible for an increased post-operative morbidity and
mortality [4, 12]. However, stents are therefore often unavoidable. The
necessary imaging should be completed to clarify the resectability before
stent insertion. A biliary stent lying complicates the intraoperative
assessment of resectability. A meta-analysis of 11 studies and a prospective
study show no significant benefit of preoperative biliary drainage [5, 21]. Not
infrequently, the patient is presented with already lying stent the
surgeon. Furthermore, can be carried out not immediately sometimes
surgery for diagnostic or logistical reasons.
A biliary stent lying complicates the intraoperative assessment of
resectability

Staginglaparoskopie
The Staginglaparoskopie has already become in many tumor types standard
in the preoperative evaluation, since minimally invasive peritoneal tumor
seeding can be diagnosed. Various working groups carry this in their routine
by [6, 10, 13]. The private general we perform laparoscopy only imaged by
advanced tumors with a high probability extrahepatic tumor growth.
By Staginglaparoskopie, a peritoneal tumor seeding are minimally invasive
diagnoses
Liver function tests
For the surgical Resektionsstrategie is the assessment of liver function and
the function of the future liver remnant tissue is of great importance. Various
Liver function tests are available, which are used particularly in the Asian
region. Their significance is disputed, in particular in the cholestatic liver. A
developed by Stockmann and employees test also seems in this case to be
able to provide additional information regarding the function of the residual
liver, has not yet been accepted as a standard [22].
Resektabilittseinschtzung
The biggest challenge for the surgeon is the assessment of resectability and
the development of appropriate Resektionsstrategie. While tumors that have
reached the same segment bile ducts of both sides, as well as tumors are
considered unresectable infiltrate both hepatic arteries. The problem is that
the intraductal linear expansion of the tumor by any method is reliably
assessed preoperatively and even the combination of different methods, the
proximal expansion often overestimated, so that in consequence of the
exclusion of contraindications only exploration permits real clarification of
resectability. This also means that not everyone as Bismuth IV classified
tumor is actually unresectable [8, 23]. The diagnostic laparoscopy has it their
priority to the exclusion of liver metastasis and peritoneal carcinomatosis,
but is not suitable for the assessment of resectability in our opinion. A
locoregional lymph node involvement does not constitute a contraindication
for surgery. Therefore, high rates have for the exploration beyond the
perihilar area bile duct carcinomas and despite optimal preoperative
diagnosis are accepted [9, 12, 16].
Only exploration permits real clarification of resectability
Surgical strategy and technique
From oncologic perspective, the R0 resection - ie free resection margins in
the proximal, distal and lateral -. The aim of the operation. The close
anatomical positional relation in particular to Pfortadergabel and the hepatic
arteries makes compliance with the usual oncologic surgery criteria ("notouch") is very difficult, and the intraoperative frozen section analysis is due

to the possible discontinuous tumor growth of limited significance and

Perineuralscheidenwachstums (that eludes this). Theoretically proximally
longitudinally a safety distance of 1-2 cm, are laterally respected by 5 mm,
this is, however, particularly in the right main bile duct considerably shorter
often impossible [10, 24]. Even with macroscopically sufficient safety margin
and thus a potentially resectable situation intraparenchymal bile ducts can
not be resected with histologically detectable unlimited infiltration, so that
there is, despite all efforts to R1 situation. After distal insufficient safety
distance can be achieved through an extension of the operation to a
pancreas.
Should longitudinally proximally a safety distance of 1-2 cm, are laterally
respected by 5 mm
Criteria of Irresektablitt
The reasons for leaving a surgical strategy may be oncological or technical
nature. Resection at present distant metastasis or peritoneal does not make
sense. Lymph node metastasis in hilar or within the range of the celiac trunk
do not constitute a contraindication to resection [10, 11, 15]. An insufficient
residual liver volume after resection is a technical problem, the z by
conditioning. B. a, portal venous embolization (PVE) can be
met [12, 25]. Resection of Pfortadergabel is technically feasible and does not
constitute a contraindication for tumor invasion. An arterial infiltration in the
area of the common hepatic artery or the artery of the remaining lobe of the
liver means a technical irresectability, although there are also here the
beginnings of a arterial reconstruction in selected patients [ 13, 26].
Hilar lymph node metastasis in or near the Tr. celiac do not constitute a
contraindication for surgery
Hypertrophiekonzept
If a Klatskin tumor type IIIa according to Bismuth, so a direct resection with
an (extended) hemihepatectomy right is usually not possible because the
Parenchymreserve the left-lateral liver segments is often too low.An
exploration is recommended in this case only at sufficiently large left lobe in
the imaging. Alternatively, of makuuchi [in this case 14, 27 for the first time
used] and Neuhaus [25 refined approach] especially for right-sided tumors
are applied. Advantage of hypertrophy in the range of segments II and III is a
prevention of postoperative liver failure [28]. For the implementation of the
PVE precise agreement between radiologists and surgeons is necessary,
especially for dealing with portovensen vascular variants from left.
Advantage of hypertrophy in the range of segments II and III is a prevention
of postoperative liver failure
The PVE is performed via a direct access to the portal system via puncture of
the right, for the embolization itself are mostly metal coils and so-called.
Plugs used [29, 30]. The decisive factor is the closure of all portals branches

to segment IV, which often depart from the left portal system. Within 3 to 5
weeks after PVE can be expected with the greatest Hypertrophieschub - with
heavily modified cholestatic liver hypertrophy may, however, analogous to
cirrhotic livers certainly take considerably longer [31, 32]. A repeat CT
diagnosis even after 8 weeks may be useful.
The decisive factor is the closure of all portals branches to segment IV, which
often depart from the left portal system
Disadvantage of the PVE is the commitment to a Resektionsstrategie. If z. B.
intraoperatively be an infiltration of the left hepatic artery, so the strategy
can not be changed on an extended left resection. Also an increased tumor
growth has been described after PVE in the remaining liver, this is, however,
relevant [more metastases of other tumors than Klatskin tumors 33].
The analysis of hypertrophy can be performed on CT data reliably together
by radiologists and surgeons. In general, we prefer to own the volumetrics by
the surgical planning software HepaVision the Fraunhofer MeVis
Group. Advantage of this type of volumetry is that as a result of a wellvascularized liver volume is calculated, which is rather equivalent to a
functional volume [34, 35]. This is particularly important with regard to the
change in operating strategy intraoperatively helpful because a segment
oriented volumetrics exists that takes into account the variation venous
drainage. For a resection in Klatskin tumors a residual liver volume of 40%
should be sought, as will be operated here in a previously damaged by
cholestasis liver.
For a resection in Klatskin tumors a residual liver volume of 40% should be
sought
A new alternative to the PVE, the two-stage operation strategy through a insitu split liver is, the so-called ALPPS ("Associated liver partition and portal
vein ligation for staged hepatectomy") -. Approach [36], the principle also at
Klatskin can be used tumors [36]. However, combining in this case the two
surgical techniques highest level of difficulty, which should be applied in view
of the already increased mortality and morbidity of this patients group only
in exceptional circumstances [37].
Resection strategies
The spectrum of Resektionsmglichkeiten ranges from the local resection of
the intrahepatic bile ducts with Gallenwegsresektion on Mesohepatektomie
as local hilar resection of the hepatic parenchyma to Hemihepatektomien left
or right. Many writers always do a segment I-resection [15], since the
segment I-bile ducts open directly in the bile duct and fork as a tumor growth
direction Segment I is likely. If the segment-I resection is feasible in view of
the residual liver volume, this should also be done.
If the segment-I resection is feasible in view of the residual liver volume, this
should also be done

Primarily be to increase the lateral safety distances in modern

times Advanced Resektionsformen(Trisegmentektomie right or left) carried
out under entrainment of the portal bifurcation in addition to resection of the
bile ducts. Due to the Perineuralscheidenwachstums of tumors and the
spread occurring with "skip lesions" the significance of intraoperative frozen
section diagnosis is very limited. This calls for the extended resections, since
only so maximum safety distances can be achieved (Fig. 4). Also, a distal
extension by a pancreas must already be included in the preoperative
strategy, as this is sometimes the only way to a R0 resection.

Fig. 4
Macroscopic representation of Klatskin tumor on resected. You can see a
significant change in the bile duct epithelium (s. Mark) in the area of the
tumor, the distal bile duct epithelium shows regular (pictured above), by the
liver resection and direction parenchyma of the safety distance is maintained
Hilar complicated bile / Mesohepatektomie
A hilar resection is hardly feasible under curative aspect, since the long-term
results from large series forinsufficient radicalism speak. In a Klatskin tumor
type Bismuth I it may be sufficient under certain circumstances. However,
the local resection has a role in treating patients who z. B. benefit due to preexisting conditions or contraindications to major hepatic resection of a
palliative resection of the bile ducts within the meaning of quality of
life. When a tumor Bismuth type 2 would be at least one segment I-resection
should be considered, but better to increase a suprahilre resection in terms
of Mesohepatektomie to the safety distances. The Mesohepatektomie
corresponds to a more radical form of Hilusresektion. Due to the preparation
above the portal bifurcation the radical nature of the difficulty of resection is
limited, however, very high. The reconstruction of the bile ducts on both
sides is a challenge, so this resection is only useful in exceptional cases
when tumors in early stages.
A hilar resection is under curative aspect hardly feasible
(Advanced) hemihepatectomy left
With a hemihepatectomy left and an extended hemihepatectomy left a
curative resection in patients succeed especially if the tumor vorwchst into
the left bile duct system. The by Nimura [38 propagated] strategy has the
particular advantage that the right-lateral liver segments usually guarantee
a sufficient residual liver volume. A segment-I resection is always part of the
strategy. Grows the tumor, however, up to the right Gallenwegssytem ago,
the maximum attainable lateral safety margin due to the necessary curve of
resection and the right existing earlier bifurcation of the biliary tract is
limited. The reconstruction of the bile ducts in this case is also usually very

complex, there must be at least 2, but often 4 segmental bile ducts

connected in an anastomosis (Fig. 5). With extended left hepatectomy is the
most technically demanding resection ever. Obtaining the venous drainage
of the right-lateral segments may in this case be a challenge, since the
middle hepatic vein has to be sacrificed in the resection.
The right-lateral liver segments usually guarantee a sufficient residual liver
volume
With extended left hepatectomy is the most technically demanding resection
ever

Fig. 5
Trisegmentektomie left - Situs after resection. Route the connection are
marked with probes biliary
(Advanced) hemihepatectomy right
Due to the anatomy of the biliary tree fork with a much longer history of the
left bile duct is the largest longitudinal and lateral safety distance in the
extended hemihepatectomy right can theoretically (Trisegmentektomie right
segment IV-VIII plus I) achieve, since in this case the bile duct is issued left
the hilar level , This requires a sufficient size of the left-lateral segments,
which may need to be augmented before. If the resection combined with a
Pfortadergabelresektion, this is the only strategy that a "no-touch"
technique allows the tumor resection, as the main tumor mass is not
tampered with during resection (Fig. 6).The oncological results of the Berlin
Working Group are equipped with a 5-year survival of 65% at application of
this radical strategy exceptionally well [24]. Nevertheless, a small tumor can
certainly also an anatomical hemihepatectomy law may be sufficient to
achieve an R0 resection - this has to be decided intraoperatively.
The extended right hepatectomy of the largest longitudinal and lateral safety
distance can be achieved

Fig. 6
Trisegmentektomie right at Klatskin tumor type IIIa. The bile duct is distally
severed and pulled on the thread side, the infiltration of Pfortadergabel is
clearly visible. The intraparenchymal surgical margins left is tumor-free. The
tumor can be removed with a Pfortadergabelresektion in a no-touch
technique en bloc with the liver now
Palliative hepatojejunostomy

Is in the exploration impossible resection, there is a surgical option for

palliation by investing a hepatojejunostomy on the liver segments III and V.
Here, by "exposing" of bile ducts in these segments onethere sufficient
drainage can be achieved directly on the liver parenchyma a spacious
anastomosis what the patient an omission regular stent exchange and
potentially less infectious complications draws (Fig. 7).Palliative radiotherapy
the tumor region is this reconstruction not in the way and is applied by us in
individual cases.

Fig. 7
Palliative scale hepatojejunostomy a Roux-loop on the right (segment V) and
left (segment III) liver lobe
Intraoperative radiotherapy
Intraoperative radiation the tumor region with a targeted volume is a
possibility of additive palliative therapy for unresectable tumors, but is rarely
available [due to the necessary technical requirements 39]. Whether
intraoperative radiotherapy (IORT) may mean even with resectable tumors,
an improvement of survival, is currently not available. With the technical
advances in stereotactic radiotherapy, the effect of IORT in the future can
certainly also achieved postoperatively, this, there are still no suitable
randomized data.Irradiation of the biliary anastomosis also appears
postoperatively safe [40].
The IORT is a possibility of additive palliative therapy for unresectable tumors
Reconstruction of the bile ducts
The reconstruction of the bile ducts is carried out by a disabled jejunum by
Roux. An accurate representation of all bile ducts to be connected is
necessary with probes, this can be simplified. For the anastomosis itself
offers a single-button technology with PDS 4/0 or 5/0 threads of strength. We
prefer whenever possible the anastomosis of all biliary tract together
as "Blanket" -Anastomose. The threads can thereby be fundamental part
directly in the parenchyma. The endoluminre splinting the anastomosis is
controversial. A valid data location does not exist to some workgroups use
principle [41]. Our own experiences suggest to drain the anastomosis
sufficient. This is done by splinting with transhepatic drainage, which can be
either "lost" or used as a classic endless drainage. With this technique,
complications in the area of the bile duct can be reduced to a minimum,
small gall leaks can heal over the drainage.
The reconstruction of the bile ducts is carried out by a disabled jejunal Roux
Conclusions for clinical practice

The treatment of patients with central or beyond the perihilar area bile duct
tumors constitutes a major challenge for the interdisciplinary treatment
team. These patients should be supplied only at a specialized
center. Preoperatively, the challenge is to develop a surgical strategy, this
has a direct influence on the nature of the preoperative conditioning of the
patient. As a diagnostic algorithm makes sense:
1. 1.
Contrast medium sonography,
2. . 2
MRI / MRCP,
3. . 3
triphasic CT,
4. . 4
ERCP / PTCD.
Despite expansion of the diagnosis the true extent of the tumor can be seen
only during a laparotomy, only intraoperatively may be decided on the
resectability. Thus, a high rate of exploration must be accepted. In the case
of the extended resection resections are preferable because only so the best
safety distances can be achieved. The Trisegmentektomie right with
Pfortadergabelresektion promises theoretically the best oncological results,
but is mainly used for right-sided tumors of importance and has the
disadvantage that almost the detour must be gone over the
Hypertrophiekonzept always. The Trisegmentektomie left is the most
technically demanding resection, but can achieve good oncological results
speak especially when left-sided tumors. Because of cholestatic starting
position before surgery and usually always present bacterial contamination
of the bile ducts after their discharge treating Klatskin tumors with a
significantly higher morbidity and mortality than usual in liver surgery is
fraught usual.
CME questionnaire
After which the classification beyond the perihilar area bile duct
carcinomas are classified?
A classification for perihilar tumors does not exist.
Several classifications are used for anatomic classification of bile duct
carcinomas beyond the perihilar area, but there is no uniform classification.
The classification of beyond the perihilar area bile duct tumors is based on
the Bismuth-Corlette classification can be distinguished in which 6 types.

The classification of beyond the perihilar area bile duct tumors is based on
the Bismuth-Corlette classification can be distinguished in which 5 types.
The classification is according to the Child-Pugh classification.
In the preoperative diagnosis of a tumor beyond the perihilar
area ...
is usually sufficient, a CT imaging to assess the resectability.
MRI is the imaging of choice.
the ultrasound examination of the abdomen is considered the "gold
standard".
a contrast-enhanced MRI should be performed.
ERCP is the ultimate diagnostic tool for assessment of tumor extent.
In a present preoperatively jaundice ...
accepts this - even during long existence - not affect the liver function.
must be a relief of cholestasis by ERCP stent.
, the decision from the stenting of the level of bilirubin.
stenting is usually not necessary, since there is no cholestasis in beyond the
perihilar area bile duct tumors.
there is no possibility for relief of cholestasis by ERCP and stent implant in
beyond the perihilar area bile duct tumors.
With regard to the assessment of resectability in Klatskin tumors ...
this preoperatively usually no problem.
is often incorrectly classified in the preoperative diagnosis, the tumor extent.
is a diagnostic laparoscopy always indicated and necessary.
is necessary preoperative biopsy to confirm the diagnosis.
is well defined in preoperative imaging vascular infiltration.
For beyond the perihilar area bile duct tumors is no contraindication
to surgery:
Locoregionally enlarged lymph nodes.
Peritoneal tumor seeding.
Preoperative pulmonary or hepatic metastases presentable.
An extensive infiltration of both lobes of the liver.
One infiltration of the hepatic artery.
Which of the following factors is no criterion of irresectability?
A too low forecasted postoperative residual liver volume in the first imaging.

Infiltration of both hepatic arteries.

Pfortadergabelinfiltration.
Tumor extension of type IV according to Bismuth.
Future residual liver volume 30%, bilirubin 15mg / dl.
The so-called. ALPPS Approach ...
is not considered a surgical procedure available in the therapy perihilrer bile
duct tumors.
is not feasible in Klatskin tumors due to the anatomical circumstances.
is beyond the perihilar area in tumors currently no established method
represents, but is feasible in selected cases to achieve a R0 resection.
is the method of choice in the surgical treatment of Klatskin tumors.
is indicated only for tumors of type I and II according to Bismuth.
In the intraoperative assessment of the resectability of a tumor
Klatskin ...
applies a venous infiltration as a Irresektabilittskriterium.
this is still not affected by a venous infiltration.
means the infiltration of the Pfortadergabel irresectability.
is only in very rare cases, before a vessel infiltration.
apply multiple metastases of both liver halves not as a criterion of
irresectability.
Regarding the Resektionstrategie ...
can be achieved with the so-called. Mesohepatektomie insufficient
radicalism.
provides a good but technically demanding alternative to resection of
Klatskin tumors of type I or II according to Bismuth is the Mesohepatektomie.
is the extended right hepatectomy at Klatskin tumors no meaningful surgical
treatment option
is the extended hemihepatectomy left at Klatskin tumors of the type IIIa
according Bismuth the method of choice.
is the extended right hepatectomy at Klatskin tumors of type IIIb according
Bismuth the method of choice.
Klatskin tumors ...
are afflicted with a good prognosis.
can be treated despite a very demanding surgical treatment in any general
surgical department.

are tumors that are well-resectable and usually accompanied by a low

perioperative morbidity and mortality.
are usually early clinically manifest by pain.
should be treated only in specialized and interdisciplinary liver surgical
centers.
Thanksgiving
The authors are of the artist Prof. Giuliana Brogi (Siena, Italy) for the
preparation of Fig. 1 extremely grateful.
Compliance with ethical guidelines