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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5762_Библиотеки_им_академика_М_И_Перельмана.pdf
158
e
Q. Lu et al.
f
g
Fig. 6.11 (continued)
• There are variable enhancement patterns in portal and
delayed phases [23].
– In portal phase, most of the lesions showed iso-
showing arterial phase heterogeneous hyperenhancement
and isointense or hypointense in portal venous and
delayed phases (Fig.6.10).
attenuating (Fig.6.10) or hyperattenuating compared
to peripheral liver parenchyma.
– In delayed phase, iso-attenuating is manifested as the
main appearance.
• It has been reported that central stellate scars in lesions
6.3.2.5 Nuclear Medicine Findings
Some previous studies reported that f-HCC showed uptake
of 99mTc labeled erythrocytes in the arterial phase followed
by wash-out on the delayed phase.
showed non-enhancement in arterial and portal vein
phases on contrast enhanced CT (Fig.6.10).
6.3.2.6 Imaging Recommendations
• Gold standard
6.3.2.4 Magnetic Resonance Imaging Findings
• On Magnetic Resonance Imaging (MRI), f-HCC is
hypointense on T1-weighted image (WI) and hyperintense on T2WI.
• On both T1WI and T2WI, the central scar is hypointense [23].
• The f-HCC lesion shows restricted diffusion on diffusionweighted imaging.
• Calcication is rarely detected inside the lesions on MRI.
• After gadolinium contrast medium was injected, the
enhancement pattern was similar to that of CT scan,
– The diagnosis of f-HCC can be conrmed by patho-
logical diagnosis after surgery or ultrasound-guided
biopsy [24].
• Protocol Advices
– As most f-HCC patients have no symptoms, B mode
ultrasound can be selected as an available tool for routine early detection and postoperative follow-up
examination.
Examination after fasting for 6–8h helps to reduce
the interference of gastrointestinal gas.
a
6 Rare Malignant Liver Tumors
159
– It is difcult to make a denite diagnosis of
f-HCC. Careful assessment of imaging modalities
before surgical operation is essential.
6.3.3 Dierential Diagnosis
6.3.3.1 Focal Nodular Hyperplasia
The central scar of focal nodular hyperplasia (FNH) is rarely
calcied, and whether there was scar had nothing to do with
the size of the lesion [25].
• FNH is rich in blood vessels, which lead to obvious
hyperenhancement in arterial phase, except for the center
scar. In portal and delayed phases, most lesions were
slightly hyperenhancement or isoenhancement.
– Except for the central scar, the enhancement of FNH
was homogeneous.
– Delayed enhancement of scar is the characteristic
manifestation of FNH.
• On MRI, the central scar of FNH is mainly hyperintense
on T2WI due to biliary ductules, while that of f-HCC is
usually hypointense, which is brous.
• In addition, FNH is iso-enhancement in hepatobiliary
phase on enhanced MRI using a liver-specic contrast
agent, but it is not common in f-HCC.
6.3.3.2 Hepatocarcinoma
• HCC mainly occurs in elderly patients with obvious gender preference (more in men over 60 years old).
• HCC has high-risk factors, including aatoxin B1 intake,
chronic viral infection of hepatitis B and C, alcohol abuse,
b
c
Fig. 6.12 A case of brolamellar hepatocellular carcinoma (f-HCC). B
mode ultrasound (BMUS) showed a large heterogenous hypoechoic
lesion in the right lobe of liver (arrows) (a). Filiform blood signals
could be detected inside the lesion (b). Arterial Doppler spectrum with
high resistance index (RI) as 0.84 was measured (c). Superb microvascular imaging revealed abundant branched blood signals inside the
lesion (d). On contrast enhanced ultrasound (CEUS), the lesion showed
arterial phase heterogeneous hyperenhancement (arrows) with central
d
acoustic shadow (e, f, g, h). The lesion showed isoenhancement in portal venous (arrows) (i) and hypoenhancement in late phase (j). On MRI,
the lesion was hyperintense on T1-weighted image (WI) (k) and hyperintense on T2WI (l). The central scar was hypointense on T1WI and
hyperintense on T2WI. The lesion showed heterogeneous hyperenhancement in arterial phase (m) and persistent enhancement in portal
venous and delayed phases on contrast enhanced MRI (n, o)
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Q. Lu et al.
f
g
h
Fig. 6.12 (continued)
6 Rare Malignant Liver Tumors
161
i
j
k l
Fig. 6.12 (continued)
162
mn
Q. Lu et al.
o
Fig. 6.12 (continued)
and non-alcoholic fatty liver disease. Most liver cancer
occurs in cirrhosis, which makes cirrhosis the strongest
inducing factor [26].
• The level of serum AFP in patients with HCC was signicantly increased.
• HCC is mainly supplied by hepatic artery and enhanced
early. The characteristic of HCC enhancement is “early in
and early out.”
– The enhancement of large HCC lesions is often uneven
because of necrosis in the center.
• Most lesions in portal phase and delayed phase are of
hypoenhancement and the boundary is generally clear.
– Compared with f-HCC, typical liver cancer showed
relatively later elution in portal vein phase.
• US is the most commonly used method to diagnose the
disease.
– Small cavernous hemangiomas are mostly hyperechoic
nodules with clear boundary, while giant cavernous
hemangiomas are mixed with hyperechoic and
hypoechoic images.
• The typical enhancement feature of hemangioma in
enhanced CT imaging is nodular or annular enhancement from the periphery, gradual expansion to the center, and iso-density or high-density lling in the delayed
phase.
– However, there are also a few atypical hemangiomas,
which are signicantly enhanced in the arterial phase
scan with high density, while in the portal phase and
delayed phase scan with equal density [25].
6.3.3.3 Cavernous Hemangioma oftheLiver
• The disease is most common in adults aged 30–50 years.
• Most patients have no clinical symptoms. The development of tumor is slow and the course of disease can last
for decades.
• On MRI, the most signicant feature of cavernous hemangioma is hyperintensity on the T2-weighted image,
showing “bulb sign.”
– If angioma has brosis or cystic change, the signal
intensity may be uneven.
6 Rare Malignant Liver Tumors
163
6.3.3.4 Hepatoadenoma
• Hepatoadenoma is a rare benign tumor in the liver, which
is mostly seen in childbearing women.
• It is related to oral contraceptive.
• Hepatoadenoma is usually a single abundant blood supply
lesion, often with capsule and intratumoral hemorrhage.
• Contrast enhanced CT showed signicant hyperenhancement in arterial phase and iso-density on the subsequent
phase images.
• Hepatoadenoma can be isointense on T1-weighted image
or a high signal caused by fat content, which greatly differs in T2 signal image, and often show signal loss.
6.3.3.5 Intrahepatic Cholangiocarcinoma
• In the early stage of ICC, there was no obvious symptom,
and the mass of liver was found by imaging when the liver
function was abnormal.
– Even though the tumor size has reached 5–7cm, about
one-third patients have no clinical symptoms.
– Nonspecic symptoms include abdominal discomfort,
vomiting, night sweats, fatigue, and others.
• On B mode ultrasound, the typical ICC shows a heterogeneous mass with irregular shape and unclear boundary,
and dilated biliary ducts may also be seen. The blood ow
of the artery with high resistance can be measured in the
focus.
• The manifestation of ICC in CEUS is similar to that of
HCC, which is “fast in and fast out,” but the elution time
is earlier than HCC.
– The border of the tumor in portal stage is clearer than
that in the arterial stage, which is benecial to the
observation of the lesion range.
• On plain CT scan, low-density intrahepatic mass without
capsule, unclear margin, and uneven density are common
manifestations.
• In dynamic contrast-enhanced imaging, the enhancement
degree of the ICC lesions depends on the local blood supply, intra-lesion necrosis, and brosis degree.
– Small ICC might show hypervascular, which is similar
to HCC imaging features during arterial phase.
– Large ICC often shows peripheral rim-like hyperen-
hancement with a central area of low attenuation.
• Plenty of brous tissue in ICC and peripheral neovascularity results in delayed hyperenhancement in the portal
venous phase.
• ICC shows low signal on T1-weighted image. On
T2-weighted image, heterogenous mass of the peripheral
shows high signal and central low signal [25].
6.3.4 Pathology
6.3.4.1 General Features
Microscopic pathology
• The microscopic characteristics of f-HCC are large eosinophils, prominent nucleoli, and intratumoral brosis [27].
– Under the microscope, the cancer cells were polygo-
nal, eosinophilic, and large nuclei. The tumor cells are
large in size, nearly three times larger than the surrounding cells.
– The tumor cells are arranged into nests, and a large
number of parallel lamellar brous tissues appear
between nests [26].
The lamellar brous tissue is arranged orderly, and
the tumor cells are divided into strips or clusters,
which have certain characteristics.
Fibrous may merge and form a central scar.
Immunohistochemistry
• f-HCC often stains strongly for CK7, CD68, and epithelial membrane antigen [28].
• Laboratory tests showed that DCP, vitamin B12 receptor,
and nervous tension value increased.
– Serum DCP was mostly used in the diagnosis and
monitoring of HCC. In 64–100% of patients with
f-HCC, the level of serum DCP is increased.
• Immunohistochemical prole similar to that of hepatocellular carcinoma, such as positive staining for hepatocyte
parafn 1 (HepPar1), glypican-3 (GPC3), arginase-1,
polyclonal carcinoembryonic antigen (pCEA), and CD10
positivity [29].
Molecular genetics
• DNAJB1-PRKACA drives f-HCC tumorigenesis in
greater than 95% of cases.
6.3.4.2 Staging, Grading, andClassication
• Grade
– Grade I: Well differentiated
– Grade II: Moderately differentiated
– Grade III: Poorly differentiated
– Grade IV: Undifferentiated; anaplastic
• TNM staging
– T1: No vascular invasion
– T2: Vascular invasion
– T3: Invasion of a major branch of the portal or hepatic
vein
– T4: Invasion of adjacent organs
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Q. Lu et al.
– N0: No local lymph node involvement
– N1: Local lymph node involvement
– M0: No metastasis
– M1: Metastasis
• Stage
– Stage 1: A single tumor of any size and no vascular or
lymph nodes invasion
– Stage 2: Single tumor, less than 5cm in size, with vas-
cular invasion but no lymph node involvement
– Stage 3: Multiple tumors, tumors larger than 5cm and
involving major blood vessels or any T-phase and positive lymph nodes
– Stage 4: Metastasis
6.3.5 Clinical Issues
6.3.5.1 Presentation
• f-HCC has a unique preference for adolescents and young
people, more women than men.
• The symptoms include abdominal pain or abdominal dis-
comfort in the upper right quadrant, nausea, weight loss,
palpable abdominal mass, and hepatomegaly [29].
• f-HCC is usually single, with good differentiation and
slow growth.
• Incidence of lymph node involvement of f-HCC patients
is higher than that of HCC patients, which may be due to
the large median tumor size. The diameter of the tumor is
usually more than 10cm [28].
• All patients’ hematological studies and liver function
tests, including transaminase, bilirubin, and
γ-glutamyltranspeptidase are within the normal range or
slightly elevated levels.
• The level of tumor marker α-alpha fetoprotein (AFP) in
f-HCC patients is usually normal.
– In general, most tumor markers have no predictive
value for the diagnosis of f-HCC.
• Compared with HCC, 95% of f-HCC occurs in patients
without any signs of hepatitis, cirrhosis, or hepatic metabolic dysfunction [30].
• Distant metastasis is found in 20–30% of f-HCC.
– Lung, peritoneum, and adrenal gland are the most
common sites.
giovascular invasion, nodule and distant metastasis, and
integrity of resection [29].
– It has been reported that the initial tumor stage before
treatment is the most important determinant of
prognosis. Patients in stage I-III tend to have a better
prognosis than patients in stage IV.
• Long-term surveillance is essential because recurrence
and death may occur several years after treatment [30].
6.3.5.3 Treatment
• Surgical resection is the main curative treatment for
f-HCC.Complete periportal lymphadenectomy is recommended to patients of f-HCC with lymph node involvement [24].
• Liver transplantation is only considered in unresectable
f-HCCs.
– As a result of extensive liver involvement, up to 30%
of cases are diagnosed without consideration of
resection.
– There is little data on the outcome of liver transplanta-
tion in f-HCC patients. Studies have shown that liver
transplantation can achieve good results in some
f-HCC patients.
• Other therapies commonly used for HCCs, such as chemotherapy, external radiation therapies, or specic drugs,
have proven ineffective for f-HCCs [23].
– For patients with advanced or recurrent invasive
f-HCC, chemotherapy with gemcitabine, cisplatin,
5-uorouracil, interferon, and oxaliplatin showed different degrees of response.
– There was an attempt to reduce the tumor with radio-
active embolization before operation.
– A new targeted therapy, sorafenib, was evaluated retro-
spectively in f-HCC and showed limited efcacy.
• Other studies have shown that multimodal treatment,
including resection, systemic chemotherapy, and radiotherapy, improves prognosis.
6.4 Hepatic Biliary Cystadenocarcinoma
YiDong, Jia-YingCao, and Wen-PingWang
6.3.5.2 Prognosis
• Despite the advanced stage, about 70% of f-HCC patients
received surgical treatment, and the overall 5-year survival rate was 70% [24].
– Patients who received lesions resection had better sur-
vival than those HCC patients.
• Although sex, tumor size, and atypia seem to be unrelated
to survival, cirrhosis is recognized as a poor prognostic
factor.
• Other prognostic factors include age, disease stage, diversity, tumor thrombosis, microvascular invasion, lymphan-
6.4.1 Terminology
Denitions
• HBCT derives from the biliary epithelium and usually
grows within the liver tissue (85%) and occasionally
within the extrahepatic biliary tract [31].
• HBCT is reported to represent only 5% of all cyst lesions
in the liver but its actual incidence is likely much higher
because of the misdiagnosis [32].
a
6 Rare Malignant Liver Tumors
165
• Mucinous cystic neoplasm, the unique subset within the
hepatic cyst differential and including the subgroup of
mucinous cystic neoplasm, is also referred to as hepatic
biliary cystadenoma and HBCAC [33].
• HBCAC is mostly malignant transformed from the
hepatic biliary cystadenoma and may also come from
glands or pluripotent stem cells around the bile ducts
[31].
• A malignant transformation rate of up to 30% has been
reported, and HBCAC accounts for about 0.41% of malignant hepatic cystic tumor tumors [34, 35].
6.4.2 Imaging
6.4.2.1 Conventional Ultrasound Findings
• HBCAC is a well-dened unilocular or multilocular cystic or cystic-solid lesion with mural or septal nodule and
papillary projections.
• A nodule diameter >10 mm and the calcications along
the wall and internal septum on conventional B mode
ultrasound are suggestive of HBCAC (Fig.6.13a).
• A ductal dilatation upstream to cystic lesion may be seen
in HBCAC (Fig.6.13b).
• HBCAC contains more solid components than HBCA
(Fig.6.14a).
• Color Doppler imaging can detect rich blood ow in septal nodules and cyst wall.
6.4.2.2 Contrast Enhanced Ultrasound Findings
• Presence of one or more of the following structures of
HBCAC can be shown in contrast enhanced ultrasound:
papillary projections, wall thickness irregularities, mural
nodules, coarse calcications along the wall, and internal
septations with nodular areas.
• HBCAC shows hyperenhancement or iso-enhancement of
the cyst wall, internal septation, mural nodule, or solid
component in arterial phase (Figs.6.13c, d and 6.14c, d).
b
c
Fig. 6.13 A case of hepatic biliary cystadenocarcinoma (HBCAC)
lesion in a 77-year-old man. Several calcications can be detected
inside the hypoechoic lesion (a). Dilated and twisted bile ducts can be
observed beside the lesion (b). On contrast enhanced ultrasound, the
irregular thickened cyst wall was observed hyperenhancement during
d
arterial phase (c, d) and rapid wash-out in portal venous phase (e). On
MRI, the lesion exhibited low signal intensity on T1-weighted image (f)
and heterogeneous high signal intensity on T2-weighted image (g). It
showed heterogeneous peripheral enhancement in arterial phase of
MRI, and continuous enhancement in portal venous and late phases
166
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Q. Lu et al.
f
g
h
Fig. 6.13 (continued)
i
a
6 Rare Malignant Liver Tumors
167
• Hypoenhancement of the cystic wall, septations, and/or
mural nodules during portal venous and late phases is the
characteristic of HBCAC (Figs.6.13e and 6.14e, f).
6.4.2.3 CT Findings
• HBCAC appears as a well-dened, unilocular or multilocular hypo-density mass, which has numerous internal
septations in variable thickness.
• Tiny calcication along the internal septa and mural nodules is more often seen in HBCAC than in HBCA.
• Papillary excrescence, nodular thickening of internal septa,
and mural nodules are signicant contrast enhancement in
HBCAC after intravenous contrast administration.
6.4.2.4 MRI Findings
• HBCAC shows as cystic hypo-signal with internal septations and uneven thickness of cysts, nodular, or papillary
soft tissue signal intensity in the cyst wall on T1-weighted
images.
• HBCAC is mainly hypersignal on T2-weighted images
with internal septations, mural nodules, and soft tissues
showing as hypo-signal.
• With the difference in the presence of solid components,
protein content and hemorrhage, the internal uid within
the HBCAC lesion can show varied signal intensity on
T1- and T2-weighted images.
• The surface of the nodules was non-smooth or papillary in
MRI images.
• Internal septa, mural nodules, and soft tissues in the arterial phase of the enhanced scan are signicantly enhanced,
and the enhancement of portal and delayed phase are still
signicant.
• Magnetic resonance cholangiopancreatography (MRCP)
images show hyperintense multilocular cystic mass, the
biliary tree and possibly a ductal dilatation upstream to
cystic lesion.
• Mural nodules were several lling defects in HBCAC on
MRCP images. A ductal dilatation upstream to cystic
lesion is more often seen in HBCAC than in HBCA.
6.4.2.5 Other Imaging Findings
• PET/CT
– F-18 FDG PET/CT demonstrates intense uptake in the
marginal parenchymal region of the HBCAC.
b
c
Fig. 6.14 A hepatic biliary cystadenocarcinoma (HBCAC) lesion with
honeycomb septa hyperenhancement. An anechoic lesion with multiple
hyperechoic septa could be found on B mode ultrasound (a). No color
ow signal could be detected in the lesion (b). On contrast enhanced
d
ultrasound, the lesion showed a rapid hyperenhancement of the honeycomb septa during arterial phase (c, d), and hypoenhancement of septa
during portal venous and late phases (e, f)