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188
Q. Lu et al.
the ratio of men to women is about 2:3. The incidence of
HEHE lies in less than 1/106 in the crowd. There are no special laboratory blood test indicators. Only some patients have
abnormal liver function blood test indicators, and most
patients do not have a history of hepatitis B.Tumor markers
such as alpha-fetoprotein, carcinoembryonic antigen (CEA),
and carbohydrate antigen 199 (CA199), carbohydrate antigen 125 (CA125) is normal mostly, and very few may have a
slight increase [56, 57].
Prognosis, Treatment, and Surveillance
There are various clinical treatment methods including surgical resection, liver transplantation, hepatic arterial chemoembolization (TACE), radiofrequency ablation, and
chemotherapy. A few patients choose to follow up without
treatment and liver transplantation is preferred when there
is no epithelioid hemangioma in other parts. Due to rarity
of the tumor, the prognosis is not yet clear, but because of
its low-to-moderate malignant potential, it has a better
prognosis than malignant tumors such as hepatocellular
carcinoma and cholangiocarcinoma, which are common in
the liver.
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Rare Benign Liver Tumors
Pei-LiFan, YiDong, Wen-PingWang, andJia-YingCao
7
Abbreviations
AFP Alpha-fetoprotein
CEUS Contrast enhanced ultrasound
FNH Focal nodular hyperplasia
HAML Hepatic angiomyolipoma
HBCA Hepatic biliary cystadenoma
HBCT Hepatic biliary cystic tumor
HCC Hepatocellular carcinoma
PEComa Tumor of perivascular epithelioid cells
WHO World Health Organization
7.1 Hepatic Angiomyolipoma
Pei-LiFan, YiDong, and Wen-PingWang
7.1.1 Terminology
Denitions
According to the World Health Organization (WHO) classication of tumors of the liver and intrahepatic bile ducts (4th
ed, 2010), hepatic angiomyolipoma (HAML) is dened as a
benign mesenchymal tumor (ICD-O/SNOMED 8860/0). It is
currently regarded as a tumor of perivascular epithelioid
cells (PEComa) expressing both myomatous and lipomatous
differentiation and melanogenesis [1].
P.-L. Fan · Y. Dong · W.-P. Wang (*) · J.-Y. Cao
Department of Ultrasound, Zhongshan Hospital, Fudan University,
Shanghai, China
e-mail: fan.peili@zs-hospital.sh.cn; dong.yi@zs-hospital.sh.cn;
cao.jiaying@zs-hospital.sh.cn
7.1.2 Imaging
Imaging characteristics of HAMLs are correlated with proportions of their tissue components, especially in the lipomatous and angiomatous components. Typical imaging
characteristics of HAMLs are fat-containing lesions with
hypervascularity, obvious intratumoral vessels, and early
draining veins [2].
7.1.2.1 Conventional Ultrasound Findings
HAMLs are usually round with well-dened margin without
a true capsule. Fat components result in obviously and homogeneously hyperechoic lesions with partial shadowing or
moderately hyperechoic lesions on B mode ultrasound.
HAMLs with few or no fat cells have various ultrasound
appearances, and often manifest heterogeneous or
hypoechoic. Some HAMLs may have anechoic foci due to
cystic degeneration or hemorrhage and necrosis [3] (Fig.7.1).
Multiple punctiform, liform, or branched vascular signals are often detected in HAMLs on color ow imaging.
Some large lesions always have hypervascularity. Nodular or
vessel-like structures may be seen occasionally in some large
lesions. Circuitous vessels may be observed surrounding
some lesions. In some tumors, branches of the hepatic vein
may be seen around, being early draining veins. Majority of
HAMLs can be detected arterial ow spectrum, and the value
of resistance index (RI) is usually no more than 0.6.
7.1.2.2 Contrast Enhanced Ultrasound Findings
Classically HAMLs on contrast enhanced Ultrasound
(CEUS) demonstrate entire signicant hyperenhancement in
early arterial phase (Figs. 7.2 and 7.3). Arterial phase
branched hyperenhancement could be seen in some big
HAML lesions (Fig.7.4). Hypervascularity HAMLs always
have well-dened margin in late arterial phase. Most HAMLs
sustain hyperenhancement in the portal venous (Fig.7.3) and
show iso-enhancement in the late phase, However, 12–25%
of lesions demonstrate relative wash-out (Figs.7.2 and 7.4).
© The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd. 2021
W.-P. Wang et al. (eds.), Contrast-Enhanced Ultrasound Imaging of Hepatic Neoplasms,
https://doi.org/10.1007/978-981-16-1761-4_7
191
192
a
P.-L. Fan et al.
b
c
Fig. 7.1 Features of hepatic angiomyolipoma (HAML) on B mode
ultrasound. A homogeneous well-dened hyperechoic focal liver lesion
was detected in the left lobe of liver with posterior shadow (a). A moderately hyperechoic lesion with anechoic foci due to cystic degenera-
Arterial phase branched hyperenhancement and wash-out
are more common in hypoechoic HAMLs [4].
d
tion (arrow) in the right lobe of liver (b). A heterogeneous hypoechoic
lesion in the liver (c). A heterogeneous echoic lesion with hemorrhage
and necrosis in the right lobe of liver (d)
MRI.However, wash-out in portal venous and delayed phases
of HAMLs on contrast enhanced MRI are more common than
persistent enhancement (Fig.7.2). Some HAMLs have obvi-
7.1.2.3 CT Findings
HAMLs are usually heterogeneously low-density image on
unenhanced CT. Fat component (attenuation: −100 to
−10HU) can be seen macroscopically on CT.Most HAMLs
ously intratumoral vessels and show an draining vein during
the early arterial phase. HAMLs also show hypointensity on
the hepatobiliary phase using gadoxetic acid or gadobenate
dimeglumine (a kind of hepatocyte-specic agents).
show arterial hyperenhancement and persistent enhancement
without wash-out, although contrast wash-out can occur.
Intratumoral vessels and an early draining vein may be a
characteristic manifestation.
7.1.2.5 Other Imaging Findings
Few literatures on nuclear medicine imaging of HAML have
been reported.
7.1.2.4 MRI Findings
On unenhanced MRI, HAMLs show heterogeneous hyperintense on T2-weighted images. MRI is the most sensitive
imaging method to show fat component, HAMLs show
hypointensity on fat-suppression images or loss of signal on
opposed-phase T1 gradient echo images. HAMLs demonstrate arterial hyperenhancement on contrast enhanced
7.1.2.6 Best Imaging Protocol Advices
• Typical imaging characteristics of HAMLs are fat-
containing lesions with arterial hyperenhancement, and
obviously intratumoral vessels with an early draining
vein.
• However, some HAMLs may have little fat and some may
show wash-out on contrast-enhanced imaging [5].
a
7 Rare Benign Liver Tumors
193
7.1.3 Dierential Diagnosis
Differential diagnoses sometimes should be made in some
hepatic lesions with fat components. Malignant lesions
include hepatocellular carcinoma (HCC), metastasis, and
liposarcoma. Benign lesions include focal steatosis, postoperative packing material, HNF-1a adenomas, lipomas,
hepatic adrenal rest tumors, and teratomas. HAMLs with
obviously intratumoral vessels should be distinguished from
focal nodular hyperplasia (FNH) and hemangiomas.
7.1.4 Pathology
7.1.4.1 General Features
Angiomyolipomas are composed of a variable proportion of fat
tissue, smooth muscle (spindled or epithelioid), and thick-
walled blood vessels, which are responsible for the protean
morphological appearance. Most angiomyolipomas are single
and tumor size is variable. They are well-dened but not encapsulated. When sectioned, HAMLs usually have a homogeneous
yellow, yellow-tan, or tan appearance, due to their fat content.
Larger tumors may have grossly evident necrosis or hemorrhage. The smooth muscle is the only special diagnostic component, including epithelioid cells in sheets or spindle-shaped
cells in bundles. The epithelioid cells can be clear (sugar cell),
oncocytic, or pleomorphic. Unusual growth patterns (trabecular, pelioid, inammatory) can sometimes be seen, especially
in myomatous tumors with few or no fat cells. Extramedullary
hematopoiesis is a frequent and characteristic feature of hepatic
tumors. Immunohistochemically, the dening smooth muscle
cells are consistently positive for melanogenesis (e.g., HMB45,
Melan A, and S100) and smooth muscle (e.g., SMA, CD117,
actin, desmin, and vimentin) markers [5].
b
c
Fig. 7.2 Features of hepatic angiomyolipoma (HAML) on B mode
ultrasound and contrast enhanced ultrasound (CEUS). A homogeneous
hyperechoic lesion with partial shadowing was observed in the left lobe
of liver, with a clear margin (a). After injection of contrast agent, the
lesion showed hyperenhancement during arterial phase (b) and portal
venous phase (c). The lesion showed hypoenhancement in late phase
(d). On unenhanced MRI, the lesion showed hyperintensity on
T1-weighted imaging (e), loss of signal on opposed-phase T1-weighted
d
imaging (f), and hypointensity on fat-suppression technique (g). The
lesion showed heterogeneous hypointensity and hyperintensity on
T2-weighted imaging (h). The lesion demonstrated arterial heterogeneous hypervascularity on enhanced MRI (i), and wash-out in portal
venous and delayed phase (j, k). The lesion had prominent tortuous
vessels in the tumor. A homogeneous yellow, round with well-dened
margin, and encapsulated lesion in the liver by gross morphology (l)
194
e
P.-L. Fan et al.
f
g
h
Fig. 7.2 (continued)
ij
7 Rare Benign Liver Tumors
kl
195
Fig. 7.2 (continued)
7.1.5 Clinical Issues
7.1.5.2 Prognosis
Most HAMLs grow slowly, and have a benign biological
7.1.5.1 Presentation
The occurrence of HAMLs has female predominance
(female-to-male ratio, 2:1 to 5:1) and has a wide age range.
An association with tuberous sclerosis is recognized
(5–10%); these patients have coexistent renal tumors and
often multiple liver tumors. Most patients are asymptomatic,
behavior. Spontaneous Regression occurs rarely. Spontaneous
rupture was reported in some relatively large lesions (>10cm
in diameter). Rare malignant examples are being recognized,
with monotypic epithelioid morphology necrosis, marked
nuclear atypia, and high proliferation activity suggested as
potentially ominous features.
and the tumors are found by accident. Patients with large
lesions may feel epigastric pain or uncomfortable. Rupture
occurs rarely in large subcapsular tumors.
Most patients have no viral hepatitis infection. Serum
tumor markers including carbohydrate antigen (CA) 19-9,
carcinoembryonic antigen (CEA), and alpha-fetoprotein
(AFP) are commonly negative.
7.1.5.3 Treatment
Surgery resection is the most common therapy method for
HAMLs recently. Conventional chemoradiotherapy has no
obvious effect. Everolimus (an mTOR inhibitor) was
reported in limited case reports to be effective in reducing
tumor size for facilitating surgical resection.
196
a
e
P.-L. Fan et al.
b
c
Fig. 7.3 Features of hepatic angiomyolipoma (HAML) on B mode
ultrasound and contrast enhanced ultrasound (CEUS). A heterogeneous
hypoechoic focal liver lesion was detected in the left lobe of liver (a).
d
f
Abundant color ow signals could be detected inside the lesion (b).
After injection of contrast agent, the lesion showed complete hyperenhancement during arterial phase (c, d, e) and portal venous phase (f)
• HBCT is reported to represent only 5% of all cyst lesions
7.2 Hepatic Biliary Cystadenoma
in the liver but its actual incidence is likely much higher
because of the misdiagnosis.
YiDong, Jia-YingCao, and Wen-PingWang
• Mucinous cystic neoplasm is a unique subset within the
hepatic cyst differential and includes the subgroup of
mucinous cystic neoplasm, also referred to as HBCA and
7.2.1 Terminology
hepatic biliary cystadenocarcinoma.
• HBCA and hepatic biliary cystadenocarcinoma are rare
Denitions
• HBCT derives from the biliary epithelium. It usually
grows within the hepatic parenchyma (85%) and occasionally within the extrahepatic biliary tract.
liver tumors with unclear etiology.
• HBCA is a benign unilocular or multilocular cystic primary tumor predominantly in middle-aged women with
malignant potential.
a
e
7 Rare Benign Liver Tumors
197
b
c
d
f
Fig. 7.4 Features of hepatic angiomyolipoma (HAML) on B mode
ultrasound and contrast enhanced ultrasound (CEUS). A heterogeneous
hypoechoic focal liver lesion was observed (a). Abundant color ow
signals could be detected inside the lesion (b). After injection of con-
trast agents, the lesion showed hyperenhancement during arterial phase
(c, d, e). In late phase, the lesion showed heterogeneous hypoenhancement (f)
198
a
P.-L. Fan et al.
7.2.2 Imaging
7.2.2.1 Conventional Ultrasound Findings
• HBCA is a round or quasi-circular anechoic lesion surrounded by an echogenic capsule with sharp demarcations and HBCA can contain multiple smooth and
hyperechogenic thin internal septations (Fig.7.5).
• The absence of mural nodularity or the small mural nodules (≤1 cm) in the cyst wall or septa is suggestive of
HBCA.
• HBCA often shows cystic or solid cystic components on
conventional ultrasound.
• HBCA has thinner septa and less thick and more regular
walls than hepatic biliary cystadenocarcinoma.
• Calcications along the cyst wall and the internal septa
are rarely seen on conventional ultrasound.
• Color ow imaging can detect inadequate blood ow in
cyst wall.
7.2.2.2 Contrast Enhanced Ultrasound Features
• Enhancement of the honeycomb septa seen during the
arterial phase of contrast enhanced ultrasound might suggest HBCA (Fig.7.6).
• HBCA shows as isoenhancement honeycomb septa during portal venous and late phases of the contrast enhanced
ultrasound.
7.2.2.3 CT Findings
• HBCA is a uid attenuating cystic mass with a soft tissue
attenuating rim, internal septa, and rarely capsular calcications and mural nodularity on CT images.
• HBCA shows mild capsular and septal enhancement after
intravenous contrast administration.
7.2.2.4 MRI Findings
• HBCA signal characteristics are typical for uidcontaining cystic lesion, including homogeneous hyposignal or iso-signal on T1-weighted images and
homogeneous hyper-signal on T2-weighted images.
• There may be some variability in both T1 and T2 signals
depending on the presence of solid components, protein
content, and hemorrhage.
• The cyst wall and septa are slightly strengthened in the
enhanced arterial phase and the portal vein phase lesions
after the administration of gadolinium–DTPA.
• Magnetic resonance cholangiopancreatography (MRCP)
images possibly show hyperintense multilocular cystic
mass, the biliary tree, and a ductal dilatation upstream to
cystic lesion.
7.2.2.5 Other Imaging Findings
• Endoscopic retrograde cholangiopancreatography
(ERCP) is uncommonly utilized for HBCA, but in
selected circumstances may be helpful in obtaining tissue
samples, identifying biliary tree communications, and
extrahepatic HBCT.
7.2.2.6 Best Imaging Protocol Advices
• Conventional Ultrasound
– HBCA should be suspected when a single unilocular
or multilocular multiseptated liver cystic lesion is
diagnosed particularly in a middle-aged woman.
b
Fig. 7.5 A typical case of hepatic biliary cystadenoma (HBCA) on ultrasound. The anechoic lesion was surrounded by an echogenic capsule and
contained smooth and hyperechoic thin internal septations (a). No color ow signal could be detected inside the lesion (b)