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Chapter 3
Table3.5 • Resectability of pancreatic tumours
Resectable Borderline Unresectable
No tumour abutment
with vessel
>5-mm length
>90° tumour
abutment with
PV or SMV
of PV or SMV
contact
>180° tumour
abutment with PV
or SMV
PV/SMV
constriction or
thrombus or
Teardrop
deformation of
SMV
Any ingrowth in
SMA
No distant metastasis Distant metastasis
PV, portal vein; SMA, superior mesenteric artery; SMV, superior
mesenteric vein.
compared with patients who had no invasion of
the vein. Glanemann etal. reported a morbidity of
21–42% and mortality of 0–5.9% when pooling a
series of 1967 patients reported in the literature.
79
The 5-year survival in this study ranged from 7%
to 20%. The systematic review of Siriwardena
found no survival benefit nor disadvantage due to
complications from resection of venous structures
with invasion.
75
Size is the only characteristic of a pancreatic
tumour that can be determined preoperatively.
A review by Garcea etal.
80
used the size measured at
histopathology. In these studies the cut-off value varied,
but most studies suggested 2 cm. In these combined
studies, the median survival of patients with tumours
2 cm was 35.5 months versus 14months for larger
<
tumours. Although the studies varied in quality, this
meta-analysis concluded that size (< or >
2 cm), is an
(independent) prognostic factor for median survival
(OR = 2.52, 95% CI 1.95–3, P <0.001). One relatively
small study used size of the tumour on preoperative
imaging and found that prognosis correlated with the
size determined on CT.
Staging and assessment of patients with
pancreatic or periampullary tumours is important
because distant metastases and extensive arterial
ingrowth preclude a resection with curative intent.
A pancreas protocol CT is the most important factor
in the staging and assessment of pancreatic cancer
for the local extent as well as the presence of distant
metastases.
81
Figure3.8 • CT of patient with pancreatic head mass
with portal contact of more than 180°.
presence of dilated collateral veins. The ‘teardrop’
sign, which describes the deformity of the otherwise
round shape of the superior mesenteric vein, suggests
venous invasion. Nevertheless, there is much debate
over the exact definition of vascular involvement in
There is still some controversy concerning the
maximal degree of vascular ingrowth and
resectability. It is clear that extensive arterial
involvement precludes a curative resection. Patients
with borderline tumours with limited vascular
involvement might benefit from vascular resection
and should undergo explorative laparotomy.
relation to resectability.
Successful resection of (a part of) the superior
mesenteric vein or portal vein has been described and
could be advantageous, provided that a R0 resection
can be achieved. In a review by Ramacciato etal.,
12 series are described with a total of 399 patients;
the morbidity ranges from 16.7% to 54% and
mortality from 0 to 7.7%, with a median survival
of 13–22months and a 5-year survival of 9–18%.
Muller etal. also described 110 patients following
resection of venous invasion with a morbidity of
41.8% and mortality of 3.6%.
78
The survival was
not increased by the addition of a venous resection
with bypass, because earlier local recurrence and/
or distant metastases arose in this group of patients
Proximal bile duct tumours
77
Patients with proximal bile duct tumours generally
present with jaundice. Patients with jaundice and
a hilar stricture will either have a benign biliary
stricture or a malignancy that has obstructed the
hepatic confluence.
includes benign biliary strictures (postoperative
bile duct injury, primary sclerosing cholangitis
(PSC), HIV cholangiopathy, Mirizzi syndrome) or
other malignancies such as gallbladder cancer or
lymphoma. Diagnosis of a hilar cholangiocarcinoma
can be challenging, particularly in patients with PSC
82,83
The differential diagnosis
52
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Staging and assessment of hepatobiliary malignancies
who may have multiple stenoses and mass lesions
without significant intrahepatic biliary dilation.
Transabdominal ultrasound
Abdominal ultrasound is often the first diagnostic
study when jaundice occurs to confirm biliary
ductal dilatation, identify the level of obstruction
and exclude gallstones.
also been useful to assess vascular involvement in
patients with proximal biliary tumours.
84
Duplex ultrasound has
85
Computed tomography and
magnetic resonance imaging
Computed tomography is used to establish the
presence of a tumour, its location as well as local spread,
vascular ingrowth and distant metastases. Magnetic
resonance (MR) cholangiography is comparable to
endoscopic retrograde cholangiopancreatography
(ERCP) in the detection of biliary malignancy. An
advantage offered by MR cholangiography is that
it can identify the luminal involvement and thus
provide more accurate staging of the tumour without
cannulation of the bile ducts and risk of infection. It
allows visualisation of both the obstructed and nonobstructed ducts, and gives important information
such as the extent of tumour within the biliary
tree and in periductal tissue, vascular and nodal
involvement, lobar atrophy, invasion of adjacent liver
parenchyma and distant metastases.
86
If imaging
studies demonstrate a focal stenotic lesion of the bile
duct in the absence of previous biliary tract surgery,
an assumptive diagnosis of hilar cholangiocarcinoma
is made until proven otherwise.
83
Endoscopic retrograde
cholangiopancreatography
ERCP and percutaneous transhepatic cholangiography
(PTC) allow for tissue sampling for pathology and
collection of bile for cytology. The value of cytology
has recently been stressed;
87
however, histological
confirmation is not mandatory before surgical
exploration. In patients that require biliary drainage
preoperatively, it is unclear whether PTC is preferable
over ERCP, but this is being studied in the randomised
controlled multicentre DRAINAGE trial.
88
Positron emission tomography
PET has been shown to have a high sensitivity for
diagnosing biliary malignancy. The limitation of
the method is that patients with an inflammatory
process of the biliary tree (i.e. PSC) can have falsepositive findings. In a prospective study by Kim
89
et al.,
PET proved significantly more accurate
in identifying distant metastases compared with
CT (58% vs 0%). Other studies have confirmed
the usefulness of PET for detecting metastases not
found by other imaging,
clinical management in up to 25% of patients.
90
ultimately influencing
91
The sensitivity of FDG-PET for detecting primary
intrahepatic cholangiocarcinoma has been
estimated at 78%.
91
However, Petrowsky et al.92
found PET-CT to be more sensitive (93% vs 55%)
and specific (80% vs 33%) in detecting intrahepatic
versus extrahepatic cholangiocarcinoma.
81
Another
limitation reported by Kluge etal., despite a sensitivity
of 92% in detecting hilar cholangiocarcinoma, was
that the rate of detection of extrahepatic tumours
was dependent on the shape of the tumour.
93
Diagnostic laparoscopy and
laparoscopic ultrasound
Information on the additional value of diagnostic
laparoscopy for malignant proximal bile duct
obstruction is limited. In a pilot study from our
institution, advanced disease was diagnosed in 19
of 47 patients (40%) by laparoscopy.
meta-analysis found that the pooled analyses of
the data suggested that one in four patients with
potentially resectable hilar cholangiocarcinoma
benefited from diagnostic laparoscopy.
given the considerable heterogeneity, a trend to
lower yield in more recent studies and further
improvement of preoperative imaging over time,
the routine use of diagnostic laparoscopy is now
being questioned. Another study found that tumour
4.5 cm, bilateral portal vein involvement and
size ≥
suspected lymph node or extrahepatic metastases
on imaging were independent factors associated
with unresectability at diagnostic laparoscopy.
proposed risk score using these variables appeared
promising to improve the yield of diagnostic
laparoscopy. A more recent study of 110 consecutive
patients in our institution confirmed these findings.
Laparoscopy revealed histologically proven incurable
disease in 44 patients (41%). Of the 65 patients who
underwent laparotomy, 35 patients (54%) were
unresectable. Although laparotomy was avoided
in 41% of cases, laparoscopy was unable to assess
resectability correctly in 44% of patients. These
findings were similar to results from the Memorial
Sloan Kettering Cancer Center involving 100
patients with carcinoma of the extrahepatic biliary
98
Thirty-five patients (35%) were identified as
tree.
having unresectable disease at laparoscopy. Of the
65 patients who underwent laparotomy, a further
94
A recent
95
However,
96
A
97
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53
Chapter 3
34 tumours (52%) were unresectable, resulting in an
overall accuracy of 51%. Finally, in a series of 401
patients with hepatobiliary cancer, the highest yield
for laparoscopy was found in patients with biliary
cancer, but the study emphasised that the surgeon’s
preoperative impression of resectability was as
important as the laparoscopic staging procedure
13
Recently, a study from our centre found that
itself.
the yield has decreased to 12% in recent years due
to better preoperative imaging.
99
During staging
laparoscopy, nodal sampling, for instance of the
station 8 hepatic artery lymph node, is also feasible.
contribute to the assessment of resectability in
these patients.98 Furthermore, extensive biliary and
vascular involvement can be determined with high
accuracy (91%) using external colour Doppler
ultrasonography, as well as thin-slice contrastenhanced multislice CT.
100
The additional value of
laparoscopic ultrasonography is therefore too low
for it to be performed routinely.
Staging and assessment
ofresectability
Laparoscopic ultrasonography has not been
very useful in staging the local tumour spread of
proximal bile duct cancer. Patients with unresectable disease most often have locally advanced
tumours, but laparoscopic ultrasonography did not
Table3.6 • TNM staging system for perihilar cholangiocarcinoma
The American Joint Committee on Cancer (AJCC)
TNM staging system is most commonly used
to stage hilar cholangiocarcinoma (Table 3.6).
However, this system is based on pathology criteria
Primary tumour (T)
TX Primary tumour cannot be assessed
T0 No evidence of primary tumour
Tis Carcinoma in situ
T1 Tumour confined to the bile duct, with extension up to the muscle layer or fibrous tissue
T2a Tumour invades beyond the wall of the bile duct to surrounding adipose tissue
T2b Tumour invades adjacent hepatic parenchyma
T3 Tumour invades unilateral branches of the portal vein or hepatic artery
T4 Tumour invades main portal vein or its branches bilaterally; or the common hepatic artery; or the
second-order biliary radicals bilaterally; or unilateral second-order biliary radicals with contralateral
portal vein or hepatic artery involvement
Regional lymph nodes (N)
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis (including nodes along the cystic duct, common bile duct, hepatic
artery, and portal vein)
N2 Metastasis to periaortic, pericaval, superior mesenteric artery and/or coeliac artery lymph nodes
Distant metastasis (M)
M0 No distant metastasis
M1 Distant metastasis
Anatomic stage/prognostic groups
Stage 0 Tis N0 M0
Stage I T1 N0 M0
Stage II T2a-b N0 M0
Stage IIIA T3 N0 M0
Stage IIIB T1-3 N1 M0
Stage IVA T4 N0-1 M0
Stage IVB Any T N2 M0
Any T Any N M1
Note: cTNM is the clinical classification, pTNM is the pathological classification.
Used with the permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is
the AJCC Cancer Staging Manual, 7th edition (2010), published by Springer New York, Inc.
54
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Staging and assessment of hepatobiliary malignancies
Figure3.9 • Classification of Klatskin tumours
according to Bismuth–Corlette, type I, II, IIIa and IV. Type
I and II tumours are limited to the confluence of the right
and left hepatic duct. In type III tumours, the segmental
branches of the right or left hepatic duct are involved
(type IIIa or IIIb, respectively). In type IV tumours, the
tumour extends into the segmental branches of both
right and left hepatic duct.
and does not provide information on the potential
for resectability. Therefore, other staging systems
have been used to predict resectability and to assess
the extent of resection. The Bismuth–Corlette
classification (
Fig. 3.9) stratifies patients based on
extent of biliary involvement by tumour. In brief,
type I: tumours below the confluence of the left and
right hepatic duct; type II: tumours reaching the
confluence; type IIIa and IIIb: tumours occluding
the common hepatic duct and either the right or the
left hepatic duct, respectively; and type IV: tumours
involving the confluence and both the right and left
hepatic ducts.
The preoperative clinical T-staging system (Table3.7)
as proposed by Jarnagin and Blumgart
101,102
defines
both the radial and longitudinal extension of hilar
cholangiocarcinoma, which are critical factors in the
determination of resectability. This Memorial SloanKettering Cancer Center (MSKCC) staging system
incorporates three factors based on preoperative
imaging studies: (1) location and extent of ductal
involvement; (2) presence or absence of portal vein
invasion; and (3) presence or absence of hepatic
lobar atrophy. Criteria for unresectability include
locally advanced tumour extending to secondary
biliary radicles (that is, sectional bile ducts [right
anterior, right posterior, left lateral and left medial])
bilaterally, to unilateral sectional bile ducts with
contralateral portal vein branch involvement,
encasement or occlusion of the main portal vein
proximal to its bifurcation, atrophy of one hepatic
lobe with contralateral portal vein involvement,
or atrophy of one hepatic lobe with contralateral
tumour extension to sectional bile ducts. A recently
designed new system of staging incorporates the size
of the tumour, extent of the disease in the biliary
system, involvement of the hepatic artery and portal
vein, evidence of lymphadenopathy, distant metastases
and the putative future liver remnant (Table3.8).
Staging and assessment of proximal bile duct
tumours is difficult and usually requires a combination
of investigations including US, CT and MRI. More
invasive tests should only be performed if necessary
in order to avoid procedure-related complications and
delay of surgery. Preoperative biopsy and brush
cytology are not always reliable and should not delay
surgical exploration.
The older staging and classification systems
were of limited use in guiding preoperative decisionmaking. This led to the development of new modified
clinical staging systems which have overcome these
limitations and can aid with preoperative
decision-making.
103
Table3.7 • Jarnagin–Blumgart clinical T-staging system
101
Biliary involvement PV involvement Lobar atrophy
T1
T2
T3
Sectional bile ducts = right anterior, right posterior, left medial, left lateral. PV, portal vein.
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Hilus ± unilateral sectional bile ducts No No
Hilus ± unilateral sectional bile ducts + Ipsilateral ± Ipsilateral
Hilus + bilateral sectional bile ducts Yes/No Yes/No
Hilus + unilateral sectional bile ducts + contralateral Yes/No
Hilus + unilateral bile ducts Yes/No + Contralateral
Hilus ± unilateral sectional bile ducts Bilateral Yes/No
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55
Chapter 3
Table3.8 • Classification system proposed by DeOliveira etal.
Bile duct (B)
†
103
B1 Common bile duct
B2 Hepatic duct confluence
B3 R Right hepatic duct
B3 L Left hepatic duct
B4 Right and left hepatic duct
Tumour size (T)
T1
<
1 cm
T2 1–3 cm
T3 ≥3 cm
Tumour form (F)
Sclerosing Sclerosing (or periductal)
Mass Mass-forming (or nodular)
Mixed Sclerosing and mass-forming
Polypoid Polypoid (or intraductal)
Involvement (>180°) of the portal vein (PV)
PV0 No portal involvement
PV1 Main portal vein
PV2 Portal vein bifurcation
PV3 R Right portal vein
PV3 L Left portal vein
PV4 Right and left portal veins
Involvement (>180°) of the hepatic artery (HA)
HA0 No arterial involvement
HA1 Proper hepatic artery
HA2 Hepatic artery bifurcation
HA3 R Right hepatic artery
HA3 L Left hepatic artery
HA4 Right and left hepatic artery
Liver remnant volume (V)
V0 No information on the volume needed (liver resection not foreseen)
V% Indicate segments Percentage of the total volume of a putative remnant liver after resection
Underlying liver disease (D)
Fibrosis
Non-alcoholic steatohepatitis
Primary sclerosing cholangitis
Lymph nodes (N)
‡
N0 No lymph node involvement
N1 Hilar and/or hepatic artery lymph node involvement
N2 Periaortic lymph node involvement
Metastases (M)
§
M0 No distant metastases
M1 Distant metastases (including liver and peritoneal metastases)
†
Based on the Bismuth classification.
‡
Based on the Japanese Society of Biliary Surgery classification.
§
Based on the TNM classification.
56
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Staging and assessment of hepatobiliary malignancies
Key points
• CT or MRI are equally accurate and should be performed for staging patients with HCC or CRLM
depending on local expertise.
• Although PET-CT may provide additional information in the work-up of patients with CRLM it does
not alter the overall resection rate or survival.
• Abdominal ultrasonography is highly accurate in identifying bile duct obstruction and stone disease
but is of limited use in detecting and staging pancreatic tumours.
• CT is the investigation of choice for the diagnostic work-up and staging of pancreatic tumours.
• The accuracy of non-invasive cross-sectional imaging with CT, MRI/MRCP and EUS is superior to
the more invasive ERCP in diagnosis of malignant bile duct obstruction.
• FDG-PET plays no routine role in patients with a pancreatic mass because it is associated with a
high rate of false-negative results.
• EUS is the most accurate technique for detecting smaller tumours (<1 cm) and for differentiating
between focal pancreatitis and malignancy.
• Diagnostic laparoscopy and laparoscopic ultrasound have a limited yield in patients with CRLM and
pancreatic cancer but are useful in hepatocellular carcinoma. The role of diagnostic laparoscopy in
proximal bile duct tumours is uncertain.
• The presence of vascular invasion and hepatocellular function determine the treatment of patients
with cancer of the proximal biliary tract.
• Surgery for HPB tumours demands careful multidisciplinary preoperative assessment of risk
factors and subsequent selection of patients. This should be done in a multidisciplinary meeting
which includes HPB specialists in the field of medical oncology, radiotherapy, gastroenterology,
(interventional) radiology and surgery.
Full references available at http://expertconsult.
inkling.com
Key references
3. Floriani I, Torri V, Rulli E, et al. Performance
of imaging modalities in diagnosis of liver
metastases from colorectal cancer: a systematic
review and meta-analysis. J Magn Reson Imaging
2010;31(1):19–31. PMID: 20027569.
This study provides evidence for the use of MRI for the
detection of colorectal liver metastases.
10. Bipat S, van Leeuwen MS, Comans EF, et al.
Colorectal liver metastases: CT, MR imaging,
and PET for diagnosis – meta-analysis. Radiology
2005;237(1):123–31. PMID: 16100087.
A good quality meta-analysis that compares the most
common diagnostic modalities to detect colorectal
liver metastases.
12. Moulton CA, Gu CS, Law CH, et al. Effect
of PET before liver resection on surgical
management for colorectal adenocarcinoma
metastases: a randomized clinical trial. JAMA
2014;311(18):1863–9. PMID: 24825641.
This randomised trial shows that PET-CT compared with
CT alone does not result in frequent change in surgical
management of patients with colorectal liver metastases.
14. Jarnagin WR, Conlon K, Bodniewicz J, et al.
A clinical scoring system predicts the yield of
diagnostic laparoscopy in patients with potentially
resectable hepatic colorectal metastases. Cancer
2001;91(6):1121–8. PMID: 11267957.
A clinical tool to identify high-risk patients with
colorectal liver metastases most likely to benefit from
diagnostic laparoscopy.
29. NordlingerB, GuiguetM, VaillantJC, etal. Surgical
resection of colorectal carcinoma metastases to
the liver: a prognostic scoring system to improve
case selection, based on 1568 patients. Association
Francaise de Chirurgie Cancer 1996;77(7):1254–
62. PMID: 8608500.
A simple prognostic scoring system to evaluate the
chances of cure in patients following resection of
colorectal liver metastases.
49. Henderson JM, Sherman M, Tavill A, et al.
AHPBA/AJCC consensus conference on staging of
hepatocellular carcinoma: consensus statement. HPB
(Oxford) 2003;5(4):243–50. PMID: 18332995.
Excellent consensus statement on the staging of
hepatocellular carcinoma.
59. van der GaagNA, RauwsEA, van EijckCH, etal.
Preoperative biliary drainage for cancer of the head
of the pancreas. N Engl J Med 2010;362(2):129–
37. PMID: 20071702.
A randomised trial which shows that routine
preoperative biliary drainage in patients undergoing
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57
Chapter 3
surgery for cancer of the pancreatic head increases the
rate of complications.
69. Van HeekNT, De CastroSM, van EijckCH, etal.
The need for a prophylactic gastrojejunostomy for
unresectable periampullary cancer: a prospective
randomized multicenter trial with special focus
on assessment of quality of life. Ann Surg
2003;238(6):894–902. PMID: 14631226.
A randomised trial showing that a prophylactic
gastrojejunostomy significantly decreases the incidence
of gastric outlet obstruction without increasing
complication rates.
75. Siriwardana HP, Siriwardena AK. Systematic
review of outcome of synchronous portal-superior
mesenteric vein resection during pancreatectomy
for cancer. Br J Surg 2006;93(6):662–73. PMID:
16703621.
The largest collective report to date on portalsuperior mesenteric vein resection in pancreatectomy
showing that cure is unlikely, even with radical
resection.
81. PhoaSS, TillemanEH, van DeldenOM, etal. Value
of CT criteria in predicting survival in patients with
potentially resectable pancreatic head carcinoma.
J Surg Oncol 2005;91(1):33–40. PMID: 15999356.
CT signs of local irresectability and a tumour diameter
3 cm predict a poor survival after resection.
of >
58
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4
Benign liver lesions
Marcel den Dulk
Cornelis H.C. Dejong
Introduction
Benign liver tumours are common and are frequently
found coincidentally. Most benign liver lesions are
asymptomatic, although larger lesions can cause
non-specific complaints such as vague abdominal
pain. Although rare, some of the benign lesions, e.g.
large hepatic adenomas, can cause complications
such as rupture or bleeding.
Ultrasound, computed tomography (CT) and
magnetic resonance imaging (MRI) of the liver
are the routine imaging modalities for the liver.
Ultrasound is often a good screening investigation
and can differentiate a cystic from a solid lesion.
CT (with contrast enhancement) and MRI can be
used to study the number and size of lesions, and
often provide further characterisation of lesions.
However, even with these imaging modalities,
it may be challenging to differentiate between
a benign liver lesion, such as a hepatic adenoma,
and a malignancy, such as a well-differentiated
hepatocellular carcinoma. Liver biopsies are in
general only undertaken if there is doubt about the
diagnosis and if results of the biopsy could influence
the management strategy.
Asymptomatic lesions are often managed
conservatively by observation. Surgical resection
can be performed for symptomatic lesions or when
there is a risk of malignant transformation. The
type of resection is variable, from small, simple,
peripheral resections or enucleations, to large
resections or even liver transplantation for severe
polycystic liver disease.
This chapter will focus on the description of
benign liver lesions in the normal liver. These
lesions can be classified by their origin, as is shown
in Table4.1.
Hepatocellular liver lesions
Focal nodular hyperplasia (FNH)
General
Focal nodular hyperplasia (FNH) is the second
commonest benign solid liver tumour, with a
prevalence of approximately 0.2%.
higher incidence in females, mainly between 20
and 40 years of age, but also occurs in men and
even in children. It is a rare finding in children but
is more commonly observed after treatment for
childhood cancer, with haematopoietic stem cell
transplantation as the most important risk factor.
Because of the predominant occurrence in females
and the young age at onset, a role for female
hormones has been suggested, but a relationship
with oral contraceptives has not been clearly
demonstrated.
and less typical.
2
In men, the lesions are often smaller
3
Clinical presentation
FNH lesions are often asymptomatic and found
during imaging for unrelated reasons.
small proportion of patients experience symptoms
such as abdominal pain or a palpable mass.
up to 20%, other liver lesions are found, such as
1
It has a
4
However, a
3
In
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59
Chapter 4
Table4.1 • Classification of benign liver lesions by
their origin
Hepatocellular Focal nodular hyperplasia
Hepatocellular adenoma
Nodular regenerative hyperplasia
Dysplastic nodules
Cholangiocellular Bile duct cysts (simple or
polycystic)
Mucinous biliary cystadenoma
Bile duct adenoma (biliary
hamartoma/von Meyenburg
complexes)
Intraductal papillary neoplasm of the
bile duct
Mesenchymal Cavernous haemangioma
Lipoma
Angiolipoma
Inflammatory Hepatic abscess (pyogenic,
amoebic)
Hydatid cysts
Others Mesenchymal hamartoma
Focal fatty infiltration
Hepatic pseudotumours
a
hepatic haemangiomata or adenomas.3 Although
FNH is commonly observed, complications are
very rare; there are only a few published cases of
spontaneous rupture resulting in intraperitoneal
haemorrhage.
Diagnosis
Ultrasound is often the initial imaging
investigation when a hepatic lesion is found.
There is only a subtle difference in echogenicity
between FNH and the surrounding normal liver.
Although the accuracy of the diagnosis increases
with the use of contrast-enhanced ultrasound
and colour Doppler, ultrasound is currently not
the modality of choice for characterisation of
an FNH.
On CT, FNH is usually homogeneous and
isoattenuating to the normal liver before contrast
injection. FNH lesions are hypervascular in the
arterial phase and typically have a central scar
(hypodense).
returns to isoattenuating compared to the normal
5
liver.
5
In the portal phase, a typical FNH
In the delayed phase, hyperattenuation of the
central scar and septae are often seen.
MRI has a higher sensitivity (70%) and specificity
(98%) for FNH than ultrasonography or CT.
Typically, FNH is iso- or hypointense on T1weighted images, is slightly hyper- or isointense on
T2-weighted images, and has a hyperintense central
scar on T2-weighted images (
Fig.4.1). A typical FNH
5
6
b
c
Figure4.1 • Focal nodular hyperplasia caudal in
the right liver lobe (segment 6). On MRI T1-weighted
images (a) late phase after Primovist, FNH may be
iso- to hypointense on T1, with a hypointense central
scar. On the T2-weighted images (b) the FNH is mainly
slightly hyper- to isointense, with a hyperintense scar. A
cross-sectional image of the resection specimen of the
same patient is seen in (c), showing the central scar.
demonstrates intense homogeneous enhancement
during the arterial phase of gadolinium-enhanced
imaging and enhancement of the central scar during
later phases.
6
60
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Benign liver lesions
Pathology
FNH is typically a lobulated lesion composed of
nodules surrounded by fibrous septa originating
from a central scar in an otherwise normal liver
(Fig. 4.1).
6
On histological analysis, a classic FNH
shows nodular hyperplastic parenchyma. The
hepatic plates may be moderately thickened (two
or three cells in thickness) with normal-appearing
hepatocytes. The central scar contains fibrous
connective tissue, cholangiolar proliferation
with surrounding inflammatory infiltrates and
malformed vessels of varying calibre, including
tortuous arteries with thickened walls, capillaries
but no portal veins. Approximately 50% of lesions
show some degree of fatty infiltration, compared
to the surrounding liver. In less than 20% of FNH
lesions, the liver shows signs of steatosis.
6
The development of FNH is thought to be caused
by an injury to the portal tract resulting in the
formation and enlargement of arterial to venous
7
shunts.
This in turn causes hyperperfusion in local
arteries resulting in oxidative stress that triggers a
response from hepatic stellate cells to produce the
typical central scar.
7
Management
In general, the diagnosis can be made on MRI
or CT, and routine biopsy is not indicated.
However, when imaging is not typical or if there is
doubt about the diagnosis, e.g. with a differential
diagnosis of hepatic adenoma or hepatocellular
adenocarcinoma, a biopsy can be considered. FNH
is currently not considered premalignant.
5
In asymptomatic patients with typical features
of FNH on imaging, no further treatment or
follow-up is required. However, further evaluation
is recommended for symptomatic lesions when the
diagnosis cannot be firmly established. The current
American College of Gastroenterology guidelines
recommend follow-up with an annual ultrasound
for 2–3 years in women diagnosed with FNH
who wish to continue using oral contraceptives. In
individuals with a firm diagnosis of FNH who are
not using oral contraceptives, follow-up imaging is
not required.
The diagnosis of FNH can be made on MRI or
CT, and routine biopsy is not indicated. However,
when imaging is not typical or if there is doubt about
the diagnosis, e.g. with a differential diagnosis of
hepatic adenoma or hepatocellular adenocarcinoma,
biopsy can be an option. FNH is not a precursor of
malignancy. In asymptomatic patients with typical
features of FNH on imaging, no further treatment or
follow-up is required. However, further evaluation is
recommended for symptomatic lesions in which the
diagnosis cannot be rmly established.
7
7
Hepatocellular adenoma
General
Hepatocellular adenomas (HCA) are rare benign
hepatic neoplasms in otherwise normal livers with
a prevalence of around 0.04% on abdominal
imaging.
women of child-bearing age (2nd to 4th decade)
with a history of oral contraceptive use; they occur
less frequently in men.
oral contraceptive usage and HCA is strong and the
risk for a HCA increases if an oral contraceptive
with high hormonal potency is used, and if it is
used for over 20months. Long-term users of oral
contraceptives have an estimated annual incidence
of HCA of 3–4 per 100 000.8 More recently, an
increase in incidence in men has been reported,
probably related to the increase in obesity, which is
reported as another risk factor for developing HCA.
In addition, anabolic steroid usage by body builders
and metabolic disorders such as diabetes mellitus or
glycogen storage disease type I are associated with
HCAs. HCAs in men are generally smaller but have
a higher risk of developing into a malignancy.
found, but in a minority of patients more than 10
lesions have been described (also referred to as liver
adenomatosis).
Clinical presentation
Small HCAs are often asymptomatic and found
on abdominal imaging being undertaken for other
purposes, during abdominal surgery or at autopsy.
Some patients present with abdominal discomfort,
fullness or (right upper quadrant) pain due to an
abdominal mass. It is not uncommon that the
initial symptoms of a HCA are acute onset of
abdominal pain and hypovolaemic shock due
to intraperitoneal rupture. In a series of patients
who underwent resection, bleeding was reported
in up to 25%.
to the size of the adenoma.
(protruding from the liver) have a higher chance of
bleeding compared to intrahepatic or subcapsular
lesions (67% vs 11% and 19%, respectively, P
<0.001).
at higher risk of bleeding compared to lesions in
the right liver (35% vs 19%, P
Diagnosis
HCAs are often detected first by ultrasound during
investigation of right upper quadrant discomfort.
The high lipid content of adenomas may contribute
to the hyperechoic appearance of these lesions.
ultrasound appearance is often heterogeneic due to
haemorrhage, necrosis and fat content. Colour
Doppler ultrasound can be used to differentiate HCA
from FNH. However, the diagnosis of adenoma is
1
HCAs are predominantly found in
8,9
The association between
In the majority of patients, only one HCA is
10,11
The risk of rupture is related
12
Lesions in segments II and III are also
11
Exophytic lesions
= 0.049).
13
The
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