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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5762_Библиотеки_им_академика_М_И_Перельмана.pdf
148
Q. Lu et al.
the non-specic clinical and imaging features of inltration type. Nowadays, with the update of treatment strategies, the 5-year survival rate has been reported to reach
77–83%.
6.1.5.3 Treatment
The optimal therapy strategy for PHL is still unclear and
treatment options include surgery, chemotherapy, radiation
therapy, or a combination of these therapies. Surgical resection might be performed for patients with localized disease,
which can be completely resected, or for debulking prior to
chemotherapy [2]. Nevertheless, recurrence after surgery is
not unusual and thus PHL is chemosensitive, combined chemotherapy is recommended. For patients with multiple nodular and diffuse inltrating types, alternative treatment
options are available, including chemotherapy, radiation, or
varying combinations. The standard treatment for patients
with DLBCL is CHOP.Rituximab combined with cytotoxic
treatment after antiviral therapy may improve clinical
outcomes.
6.2 Combined Hepatocellular
andCholangiocarcinoma
Jia-YingCao, YiDong, and Wen-PingWang
6.2.1 Terminology
Denitions
• Combined hepatocellular and cholangiocarcinoma
(cHCC-CCA) is a primary liver cancer (PLC) with unique
biphenotypic presence of both hepatocytic and cholangiocytic differentiation.
6.2.2 Imaging
6.2.2.1 General Features
• The cHCC-CCA lesions are usually found in patients
with chronic viral hepatitis or liver cirrhosis. They could
be misdiagnosed as HCC for their similar imaging appearances and chronic liver disease background.
• It is imperative for radiologists to understand the imaging
characteristics of its subtypes according to the 2019 World
Health Organization (WHO) classication of tumors of
the digestive system.
• The 2010 WHO classication system classied cHCC-
CCA into three variants, including “typical” subtype,
“intermediate cell” subtype, and “cholangiocellular”
subtype.
• Contrast enhanced pattern on CEUS/CECT/CEMRI
– HCC-like pattern
– CCA-like pattern
– Mixed pattern
6.2.2.2 Conventional Ultrasound Findings
• It is convenient to detect cHCC-CCA lesions on B mode
ultrasound, but the accurate diagnosis of cHCC-CCA can
be a great challenge because of its untypical grayscale
ultrasound features.
• The cHCC-CCA lesions were demonstrated heteroge-
neously hypoechoic with unclear margins and irregular
shapes on B mode ultrasound (BMUS) [6].
• Target appearance, as a specic appearance of cHCC-
CCA, is dened as ovoid hypoechoic mass with central
hyperechoic portion (Fig.6.6) [7]. However, it is not recommended as a common pattern of this rare tumor.
• Color ow imaging may help to detect arterial ow sig-
nals and arterial Doppler spectrum with high resistance
index (RI) (Fig.6.6) [6].
6.2.2.3 Contrast Enhanced Ultrasound Findings
• As HCC-like features were more common than CCC-like
features, most cHCC-CCA lesions show similar
enhancement with HCC on CEUS.They show hyperenhancement in arterial phase and rapid wash-out [6]
(Figs.6.6 and 6.7).
– HCC-CCA could show rim enhancement (Fig.6.8) or
complete enhancement (Fig.6.9) during arterial phase,
and hypoenhancement in early portal phase.
– Early wash-out was observed in 64% lesions, whereas
late wash-out was only observed in 4% lesions.
• The enhancement pattern of cHCC-CCA varies according
to the histological variety of CCA, rather than HCC.
• Combination of enhancement patterns on CEUS and
serum tumor markers (AFP and CA19-9) may be helpful
in differentiating HCC-CCA from other primary liver
tumors [8].
6.2.2.4 CT Findings
• HCC-CCA lesions may show well-dened low and/or
isoattenuation on plain CT scan [7].
• Contrast-enhanced computed tomography (CECT) is
helpful in differentiating cHCC-CCA from HCC when
pseudocapsule and extensive necrosis exist [9].
• The presence of intermediate characteristics between
HCC and CCA leads to misdiagnosis [10].
• The cHCC-CCA showed hypervascularity on computed
tomography angiography (CTA) images.
6.2.2.5 MRI Findings
• The appearances of cHCC-CCA may differ in patients
with normal, brotic, and cirrhotic livers [11].
e
a
6 Rare Malignant Liver Tumors
149
b
c
d
f
Fig. 6.6 A case of combined HCC-CCA (HCC 50% and ICC 50%). B
mode ultrasound revealed a focal liver lesion with heterogeneous
hypoechoic in the right lobe of liver (a). Color ow imaging showed
dotted color ow signals inside the lesion (b). Arterial Doppler spectrum with high resistance index (RI) as 0.80 was measured (c). After
injection of contrast agents, the lesion showed heterogeneous hyperen-
• HCC-CCA could be detected moderate hyperintense on
T2-weighted images.
• The ratio of cellular and brous tissue within the tumor,
rather than the characteristics of the tumor cells themselves, may contribute to the enhancement pattern.
hancement in arterial phase. The hyperechoic part of the lesion on B
mode ultrasound (BMUS) showed marked hyperenhancement, while
the hypoechoic part of the lesion on BMUS showed rim-like hyperenhancement (d, e). The lesion showed heterogeneous wash-out in the
portal venous phase, and the hypoechoic part of the lesion on BMUS
showed more obvious hypoenhancement (f)
• Enhancement patterns of cHCC-CCA may differ in different tumor sizes.
• Enhancement patterns of cHCC-CCA include ringenhancement and diffuse heterogeneous enhancement in
early arterial phase.
150
a
Q. Lu et al.
• MRI could be used to differ HCC-CCA from CCA [12].
– The most common enhancement patterns for CCA
were peripheral hypoenhancement with progression,
followed by heterogeneous hypoenhancement with
progress on contrast enhanced MRI.
– The cHCC-CCA lesions could be associated with arte-
rial phase enhancement, the presence of wash-out,
gradual enhancement without wash-out, intra-lesional
fat, and hemorrhage [13].
6.2.2.6 Other Imaging Findings
• The fusion of positron emission tomography (PET)/CT
images is useful in detecting extrahepatic metastases in
HCC and HCC-CCA [14].
• The cHCC-CCA lesions combined with primary biliary cir-
rhosis showed FDG-PET accumulation of the tumor area
and lymph node and simultaneous elevation of AFP [15].
6.2.2.7 Imaging Recommendations
• Conventional B mode ultrasound could be used for early
detection and follow-up after surgery.
• The diagnosis of cHCC-CCA should be considered when
a hypovascular PLC lesion is associated with remarkable
elevation of AFP levels and multiple regional lymphadenopathy, or when a hypervascular PLC lesion is associated with increased serum levels of CEA and CA199.
• CEUS could reveal microvascularity of tumor and realtime dynamic enhancement without missing the early
arterial enhancement.
• Protocol advice
– Biopsy
US-guided biopsy is recommended as an potential
technique to make a denite diagnosis. Biopsy should
be performed when other examinations were inconclusive or atypical.
b
c
Fig. 6.7 A case of combined HCC-CCA (HCC 50% and ICC 50%). B
mode ultrasound (BMUS) revealed a hypoechoic mass near the capsule
of the right lobe of liver (arrow) (a). No color ow signals could be
d
detected inside the lesion (b). On CEUS, the lesion showed hyperenhancement in arterial phase (c, d, e, f) followed by wash-out in most of
the lesion in portal venous phase (g)
e
6 Rare Malignant Liver Tumors
g
151
f
Fig. 6.7 (continued)
– CEUS
6.2.4 Pathology
The combination of CEUS and elevation of tumor
markers is helpful to diagnose HCC-CCA.
6.2.4.1 General Features
• It is recommended that each component and its proportion should be mentioned of cHCC-CCAs, which exhibits
6.2.3 Dierential Diagnosis
wide histological diversity.
• It is a mixture of HCC, CCA, and other connective tissues
HCC and CCA
[16].
• The cell arrangement of HCC lesions are in trabecular or
• The imaging appearance of cHCC-CCA is similar to both
HCC and CCA because of its components of HCC and
CCA, which make it confusing to diagnose.
• Since histological diagnosis is the only examination to
conrm this rare tumor, US/CT guided biopsy is
crucial.
pseudoglandular formation, while the CCA cells are
arranged in duct-like formation.
• The cellular origin of cHCC-CCA still remains unclear.
• Sarcomatoid change transformation is the most common
transformation of HCC-CCA. Spindle cell carcinoma
component could be observed in tumors.
• VEGF expression is more likely to be positive in HCC
than HCC-CCA.
152
a
Q. Lu et al.
6.2.5 Clinical Issues
6.2.5.1 Presentation
• Most common signs/symptoms
– Asymptomatic
– Abdominal pain, jaundice
• HCC-CCA only conrmed from histology and immuno-
histochemistry, the real incidence may be higher than
published gures for absence of an accurate pathological
diagnosis.
• HCC-CCA characterizes in male predominance.
• HBV and HCV are likely to be the pivotal pathogenic fac-
tors [17].
• A large part of HCC-CCA with background of cirrhosis
[18].
6.2.5.2 Prognosis
• There exist etiological differences between Asian and
Western cHCC-CCA patients.
• HCC-CCA is similar to HCC in demographics and clinical
characteristics, but shows similarity with CCA in prognosis.
• The overall survival rate of HCC-CCA is lower than HCC
but higher than CCA.
6.2.5.3 Treatment
• Treatment depends on the predominant component of the
cHCC-CCA lesions.
• Radical operation is the optimal therapeutic modality [19].
– Lymph node dissection is crucially important for
prognosis.
– Postoperative recurrence limited the efcacy of
treatment.
– Radical operation is the best choice for recurrence
lesions as well.
• Local ablation therapy (LAT)
– Radiofrequency ablation, pure ethanol/acetic acid
injection therapy, and transcatheter arterial
chemoembolization.
b
c
Fig. 6.8 A case of combined HCC-CCA (HCC 30% and ICC 70%). B
mode ultrasound (BMUS) revealed a hypoechoic mass near the surface
of the right lobe of liver (a). Dotted color ow signals could be detected
d
inside the lesion (b). On contrast enhanced ultrasound (CEUS), the
lesion showed rim-like hyperenhancement in arterial phase (c, d, e),
followed by wash-out in portal venous and late phases (f, g, h)
6 Rare Malignant Liver Tumors
153
f
g
Fig. 6.8 (continued)
– LAT may be valuable for patients with recurrent small
cHCC-CCA and hypohepatia [20].
• TACE
– TACE is likely to be helpful in patients with recurrent
unresectable cHCC-CCA [20].
• Palliative chemotherapy.
– Palliative therapies are for advanced stage patients.
• Liver transplantation (LT)
6.3 Fibrolamellar Hepatocellular
Carcinoma
YiDong, Pei-LiFan, and Wen-PingWang
h
6.3.1 Terminology
Denitions
• Fibrolamellar hepatocellular carcinoma (f-HCC) is a kind
of primary liver cancer.
– The epidemiologic, clinical, and histopathologic fea-
tures of f-HCC are signicantly different from HCC
[21].
• The etiology of f-HCC is still unknown.
• The biological behavior of f-HCC is various from a lazy
tumor to an aggressive pattern (local invasion or distant
metastasis).
154
ef
a
cd
Q. Lu et al.
b
g
Fig. 6.9 A case of combined HCC-CCA (HCC 20% and ICC 80%). B
mode ultrasound (BMUS) revealed a hypoechoic focal liver lesion
under the capsule of the right lobe of liver (arrow) (a). No color ow
signal could be detected inside the lesion (b). On contrast enhanced
ultrasound (CEUS), the lesion showed entire hyperenhancement in
arterial phase (c, d, e), followed by mild wash-out in the portal venous
and late phases (f, g)
a
6 Rare Malignant Liver Tumors
155
6.3.2 Imaging
6.3.2.1 Conventional Ultrasound Findings
• f-HCC manifests as a solid and heterogeneous lesion with
capsule on B mode ultrasound (US) (Fig.6.10) [21].
• The lesions are mainly isoechoic, with small hyperechoic
areas inside, without signs of chronic liver disease or
changes in portal vein or bile duct permeability.
• Almost all f-HCC lesions occur in normal liver, rarely in
liver cirrhosis.
6.3.2.2 Contrast Enhanced Ultrasound (CEUS)
Findings
• The f-HCC lesions show arterial phase heterogeneous
hyperenhancement and hypoenhancement in portal
venous and late phases, which is a sign of malignant
tumor [22] (Fig.6.11).
– Centripetal enhancement without spoke-like images
(Fig.6.10).
• Sometimes, a non-enhanced central scar could be detected
inside f-HCC lesions (Figs.6.10 and 6.12) [22].
6.3.2.3 Computed Tomography Findings
• Computed Tomography (CT) plain scan shows low- density
mass with a clear margin and lobulation (Fig.6.10).
• It is an important characteristic of f-HCC that there is a
star-shaped brous scar in the center of the tumor
(Fig.6.10).
– A large scar (width >2cm) and radial septa are com-
monly seen.
• Another characteristic is that there may be spot calcication in the center of the scar [22].
• Most f-HCC lesions show heterogeneous hyperattenuation on arterial phase images (Fig.6.10).
b
c
Fig. 6.10 A case of brolamellar hepatocellular carcinoma (f-HCC). B
mode ultrasound (BMUS) revealed a heterogenous hypoechoic lesion
(acoustic shadow) in the left lobe of liver (a). Filiform blood signals
could be detected inside the lesion (b). Arterial Doppler spectrum with
high resistance index (RI) as 0.77 was measured (c). After injection of
contrast agent, the lesion showed heterogeneous centripetal hyperenhancement during arterial phase, and the central scar was not enhanced
d
(d, e, f, g) on contrast enhanced ultrasound (CEUS). The lesion showed
centrifugal wash-out in portal venous and late phases (h, i, j). On CT
plain scan, the lesion was a lobulated hypodensity mass with clear margin (k). On dynamic enhanced CT, the lesion showed heterogeneous
hyperenhancement in arterial phase (l) and iso- enhancement in portal
phase (m) with non-enhanced brous central scar in the center of tumor
156
e
ij
Q. Lu et al.
f
g
h
k
Fig. 6.10 (continued)
m
a
6 Rare Malignant Liver Tumors
l
Fig. 6.10 (continued)
157
b
c
Fig. 6.11 A case of brolamellar hepatocellular carcinoma (f-HCC). B
mode ultrasound (BMUS) revealed a large heterogeneous hypoechoic
lesion in the left lobe of liver (arrows) (a). Abundant color ow signals
could be detected inside the lesion (b). After injection of contrast agent,
d
the lesion showed arterial phase heterogeneous hyperenhancement followed by wash-out in portal venous and late phases (arrows) (c, d, e, f,
g)