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5 Benign Liver Tumors
137
c
e
Fig. 5.26 (continued)
d
f
is usually <40 Hounseld units (HU) or becomes more
than 10HU difference with spleen and surrounding liver
parenchyma.
• Most hypoattenuating lesions have irregular shapes without space-occupied effect, and normal hepatic vessels can
pass through the lesions [27].
5.4.2.4 MRI Findings
• FFI can be condently diagnosed when the signal intensity of the lesion decreases on opposed-phase images versus in-phase images (Fig.5.25), whereas signal intensity
of the surrounding liver parenchyma has no difference
between the two phases.
• Additional imaging features of FFIs include irregular border, and similar enhancement pattern to the surrounding
liver parenchyma [28].
5.4.2.5 Other Imaging Findings
• None
5.4.2.6 Best Imaging Protocol Advices
• Ultrasound: The imaging method of the rst choice for
the screening and surveillance of patients with chronic
liver disease.
• Doppler ultrasound:
– To evaluate the waveform of blood ow signals.
– To evaluate the presence of normal portal or hepatic
veins through the lesion area.
• Contrast Enhanced Ultrasound:
– Isoenhancement and wash-out of local hyperechoic
lesions on CEUS suggest focal steatosis of the liver.
– Isoenhancement and wash-out of local hypoechoic
lesions on CEUS suggest focal fatty inltration lesion
of the liver.
• MRI:
– Chemical shift MRI is the current gold standard for
diagnosing focal fatty inltration.
– Magnetic resonance spectroscopy (MRS) can detect
focal fat quantication accurately.
– Signal intensity of the lesion decrease on opposed-
phase images versus in-phase images.
5.4.3 Dierential Diagnosis
5.4.3.1 Hepatocellular Carcinomas
• HCCs usually show hyperenhancement in arterial phase
with a disorder vascular pattern.
• In portal venous and late phases, HCCs commonly show
hypoenhancement except that some well-differentiated
HCCs may manifest isoenhancement.
138
J.-Y. Cao et al.
5.4.3.2 Metastases
• Liver metastases can be recognized reliably as hypoenhancement during the portal venous phase with previous
history.
• Wash-out of liver metastases is often observed in early
portal venous phase, and marked.
5.4.3.3 Hemangioma
• Hemangiomas usually appear as well-dened, roundshaped hyperechoic and homogeneous focal liver lesion
(FLL) without halo sign on B mode ultrasound. Few vessels were observed inside the lesion at color ow images.
• Hemangiomas show arterial phase peripheral nodular
enhancement with progressive centripetal ll-in, partially,
or totally. The lling process lasts from seconds to minutes, but is rapid in small lesions. Isoenhancement or
hyperenhancement in the late phase on CEUS.
5.4.4 Pathology
5.4.4.1 General Features
• Fatty liver can be divided into macrovascular and microvascular steatosis.
– Macrovascular steatosis, which contains large fat vac-
uoles and displaced nuclei in the hepatocytes, is typically associated with excessive alcohol intake,
non-alcoholic fatty liver disease (NAFLD), and type II
diabetes.
– Microvascular steatosis, which is characterized by
small intracytoplasmic fatty inclusions without the displacement of the nuclei, usually occurs with severe
impairment of mitochondrial fatty acid beta-oxidation,
as either a primary disorder or a secondary to drug
toxicity.
• FFI is a form of fatty liver disease due to increased triglyceride content in the liver.
5.4.4.2 Staging, Grading, andClassication
• The etiology of FFI could be divided into alcoholic fatty
liver and non-alcoholic fatty liver.
• Pathological subtypes of NAFLD:
– Simple steatosis
– NAFLD without steatohepatitis
– Steatohepatitis
– With or without brosis or cirrhosis
• According to the percent of involved hepatocytes, steatosis is graded 0–III (0 is none; grade I is up to 33%; grade
II is 33–66%; grade III is more than 66%). Zonal distribution of steatosis and the presence of microvascular steatosis were noted.
5.4.5 Clinical Issues
5.4.5.1 Presentation
• Fatty liver disease is often associated with metabolic syndrome and obesity. It has shown a rapid increase in prevalence worldwide.
• Fatty liver disease is often due to excessive triglycerides
accumulation in hepatocytes.
• The predilection sites of FFI are usually without portal
venous supply, including the gallbladder bed, the medial
segment of the left liver lobe close to the falciform ligament, the forepart of segment I, and the back of segment IV.
5.4.5.2 Prognosis
• Mild degree of fatty liver disease is not life threatening.
• If left untreated, it could progress to a severer degree correlated with inammation, hepatic impairment, cirrhosis,
and even hepatocellular carcinoma.
• The most common death causes are cardiovascular events
rather than hepatic diseases among patients with fatty
liver disease.
5.4.5.3 Treatment
• Controlling risk factors, such as weight reduction, tight
glycemic control, and abstinence.
• When co-existing chronic liver diseases occur and diagnosis is unclear, liver biopsy is recommended to perform
in FFI patients.
• There are no specic drugs to treat fatty liver at present.
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Rare Malignant Liver Tumors
QingLu, Pei-LiFan, YiDong, Jia-YingCao,
andWen-PingWang
6
Abbreviations
18F-FDG-EPT/CT Fluorine-18-uorodeoxyglucose posi-
tron emission tomography/computed
tomography
CCA Cholangiocarcinoma
CDFI Color Doppler ow imaging
CEUS Contrast enhanced ultrasound
cHCC-CCA Combined hepatocellular carcinoma
and cholangiocarcinoma
CT Computed tomography
DLBCL Diffuse large B cell lymphoma
f-HCC Fibrolamellar hepatocellular
carcinoma
FLL Focal liver lesion
HAS Hepatic angiosarcoma
HBCAC Hepatic biliary cystadenocarcinoma
HBCT Hepatic biliary cystic tumor
HCC Hepatocellular carcinoma
HEHE Hepatic epithelioid
hemangioendothelioma
HL Hodgkin lymphoma
HNEN Hepatic neuroendocrine neoplasm
ICC Intrahepatic cholangiocarcinoma
MHNEN Metastatic hepatic neuroendocrine
neoplasm
MRI Magnetic resonance image
NEN Neuroendocrine neoplasm
NHL Non-Hodgkin lymphoma
PHL Primary hepatic lymphoma
PHNEN Primary hepatic neuroendocrine
neoplasm
US Ultrasound
6.1 Hepatic Lymphoma
QingLu, Pei-LiFan, and YiDong
6.1.1 Terminology
Denitions
• Primary hepatic lymphoma (PHL) is a type of lymphoma
that is conned to the liver without inltration of other
lymphoid structures. It is a very rare entity that was rst
reported in 1976 and accounts for less than 1% of all
extra-nodular lymphomas.
• Morphologically, there are two types of PHL: Nodular
type (solitary nodular type 60%, multiple nodular type
35%), and diffuse inltrating type without nodular formation (5%).
• Non-Hodgkin lymphoma (NHL) is the most common histological subtype of PHL with diffuse large B-cell lymphoma (DLBCL).
• PHL is chemo- and radiosensitive, thus patients’ prognosis can be beneted from early precise diagnosis followed
with multimodality therapy.
6.1.2 Imaging
Q. Lu · P.-L. Fan · Y. Dong · J.-Y. Cao · W.-P. Wang (*)
Department of Ultrasound, Zhongshan Hospital, Fudan University,
Shanghai, China
e-mail: lu.qing@zs-hospital.sh.cn; fan.peili@zs-hospital.sh.cn;
dong.yi@zs-hospital.sh.cn; cao.jiaying@zs-hospital.sh.cn
© 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_6
Morphologically, PHL is divided into three patterns: solitary
nodule (60%), multiple nodules (35%), and diffuse inltrating type without nodular formation (5%). Solitary and multiple nodules are classied as nodular type.
141
142
a
Q. Lu et al.
Diffuse type
• Diffuse type of lymphoma is reported to be a common
inltrating pattern in secondary metastatic liver involvement; however, it is relatively rare in PHL (Fig.6.1).
• On imaging examinations, either on US or on CT/MRI,
diffuse homogeneous or heterogeneous hepatomegaly is
the only manifestation, with no discrete space-occupying
lesions demonstrating.
• No portal vein thrombus or biliary dilation is detected in
the liver.
• Hepatic vessels are detected without compression, distortion, or occlusion.
• On contrast enhanced imaging (CEUS, contrast enhanced
CT/MRI), the whole liver demonstrates no abnormal
enhancement in arterial phase without wash-out in portal
venous and equilibrium (late) phases.
• Sporadic irregular hypoechoic/low-density areas may
scatter throughout the liver showing no enhancement,
which may be due to the necrosis within the lesions.
• Diffuse intense FDG uptake (homogeneous or heterogeneous)
in the enlarged liver on FDG-PET/CT in sporadic reports.
Nodular type
Focal lesion, either solitary or multiple, is the most common
PHL imaging presentation. The imaging studies of nodular
type PHL show various patterns.
6.1.2.1 Conventional Ultrasound Findings
• Echogenicity depends on the lesion size, with homogeneous hypo-echogenicity in small lesions and heterogeneous hypo-echogenicity in large lesions (Fig.6.2).
Furthermore, marked hypo-echogenicity, which may
mimic pseudo-cyst, was the characteristic US feature
of PHL, compared with that of liver parenchyma
(Fig.6.3).
• Most lesions show round with a well-dened border.
Diffused distribution with a vague margin can also be
detected.
• On color ow image, abundant peri-lesional and interlesional color ow signal can be detected (Fig.6.4).
• Penetrating sign, that is portal or hepatic vein inside the
lesion without distortion, invasion, and occlusion, is also
the characteristic imaging feature of PHL.
6.1.2.2 Contrast Enhanced Ultrasound Findings
• In arterial phase, patchy slight homo-/heterogeneous
hyper- (Figs.6.3, 6.4, and 6.5)/iso-enhancement (Fig.6.2)
and rim-like hyperenhancement are the most common
enhancement pattern. The enhanced margin may be welldened or ill-dened.
• In portal venous and late phases, hypoenhancement compared with liver parenchyma is the common wash-out pattern (Figs.6.2–6.5).
• Penetrating sign, which is portal or hepatic vein inside the
lesion without distortion, invasion, and occlusion, can
also be detected (Figs.6.4 and 6.5).
b
Fig. 6.1 Diffuse type of primary hepatic lymphoma. Diffuse homogeneous hepatomegaly on B mode ultrasound, with no discrete space- occupying
lesions demonstrated (a). Normal course of hepatic vein vessels with no distortion or occlusion (b)
a
6 Rare Malignant Liver Tumors
143
b
c
d
Fig. 6.2 Nodular type of primary hepatic lymphoma. B mode ultrasound displayed a large hypoechoic lesion with well-dened margin
and irregular shape (arrow), the hyperechoic lesion demonstrated is a
hemangioma (arrowhead) (a). On CEUS, the large lesion showed isoenhancement in arterial phase (14s), and on this image, a penetrating sign
6.1.2.3 CT Findings
• Homogeneous hypo-attenuate lesion on plain scan, with
round or oval shape and well-dened margin. Patchy
hypo-attenuation may also be uncommonly detected.
• In arterial phase, most lesions show rim-like slight
enhancement or patchy slight enhancement.
• In portal venous and delayed phases, wash-out can be detected.
Some lesions may show central delayed enhancement.
• Penetrating sign, that is portal or hepatic vein inside the
lesion without distortion, invasion, and occlusion, is also
the imaging feature PHL.
can be detected (arrow) (b). The lesion showed hypoenhancement in
portal venous (c) and late phases (d). The hemangioma next to the
lesion manifested peripheral nodular hyperenhancement followed by
gradual lling in
• In arterial phase, PHL lesions may show various enhancement patterns, with hyper-, iso-, and hypo-intense displayed. The enhancement pattern is not characteristic.
• In portal and equilibrium phases, PHL lesions may show
heterogeneous wash-out or no wash-out.
• However, most PHL lesions show hypo-intense signal in
hepatobiliary phase.
• A signal restriction can be commonly detected on the
diffusion-weighted image.
• ADC value of lesions is much lower than that of the surrounding liver parenchyma, which is characteristic for
PHL.
6.1.2.4 MRI Findings
• Hypo-intense on T1-weighted image, hyper-intense on
T2-weighted image.
• Penetrating sign, that is portal or hepatic vein inside the
lesion without distortion, invasion, and occlusion, is commonly detected.
144
a
e
Q. Lu et al.
b
c
d
Fig. 6.3 Nodular type of primary hepatic lymphoma. B mode ultrasound displayed a markedly hypoechoic lesion near the surface, which
mimicked pseudo-cyst (arrow) (a). On contrast enhanced ultrasound
(CEUS), the lesion showed hyperenhancement in arterial phase (b, c)
followed by mild wash-out in portal venous and late phases (d, e)
a
e
6 Rare Malignant Liver Tumors
145
b
c
d
f
Fig. 6.4 Nodular type of primary hepatic lymphoma. B mode ultrasound displayed a heterogeneously hypoechoic lesion in the right lobe
of liver (a). On color ow image, abundant inter-lesional color ow
signals can be detected (b). Arterial Doppler spectrum with high resis-
tance index (RI) as 0.72 was measured (c). On contrast enhanced ultrasound (CEUS), the lesion showed hyperenhancement in arterial phase
(d) followed by wash-out in portal venous and late phases, and vessels
inside the lesion were detected (arrows) (e, f)
6.1.2.5 Other Imaging Findings
• 18F-FDG PET/CT scanning can produce whole-body
imaging data and distinguish primary liver lesions from
metastatic disease.
• The hepatic lesions of PHL always show high FDG
uptake.
• For nodular type, abnormal ring-like metabolic focus at
the site of lesion with elevated FDG uptake (SUVmax
3.5–8.2, lesion-to-liver SUVmax ratio 1.40–2.91, and
lesion-to-blood pool SUVmax ratio 1.76–4.35).
• For diffuse inltrating type, diffuse intense FDG uptake
in the liver may be demonstrated (much higher than the
physiological uptake in the brain).
6.1.2.6 Best Imaging Protocol Advices
Suspicion of hepatic lymphoma based on clinical ndings
include lactate dehydrogenase elevation, unexplained abnormal liver function, viral infection statuses (hepatitis B virus,
hepatitis C virus, Epstein-Barr virus), transplant history, and
immune deciency status.
Suspicion of hepatic lymphoma based on radiological features include (1) diffuse type: homogeneous/heterogeneous
hepatomegaly with normal course of hepatic vessels without
compression, distortion, or occlusion, together with no abnormality in contrast enhanced imaging; (2) Nodular type: patchy
arterial enhancement, delayed enhancement in dynamic phase,
vascular penetrating sign, signicantly low ADC.
146
a
Q. Lu et al.
b
c
d
Fig. 6.5 Nodular type of primary hepatic lymphoma. B mode ultrasound displayed a slightly hypoechoic lesion with an ill-dened margin
and an irregular shape (a). On contrast enhanced ultrasound (CEUS),
the lesion showed patchy hyperenhancement in arterial phase with an
US-guided core needle biopsy is the next step for the
diagnosis. Immunohistochemistry, gene rearrangement,
karyotyping, and ow cytometry examination for the biopsy
sample are essential to diagnosing histological types.
Because the PPV of this examination is extremely high,
97.1% of the diagnosis as lymphoma is very reliable. Then
18F-FDG PET/CT should be performed to distinguish primary and secondary hepatic lymphoma, and to establish the
staging of lymphoma for determining the therapeutic strategies [1, 2].
ill-dened margin (b). The lesion showed hypoenhancement in portal
venous and late phases (arrow) with an ill-dened margin (58, and
120s, respectively), and penetrating sign could be detected (arrow) (c,
d)
Hepatomegaly, either homogeneous or heterogeneous, has
no high sensitivity and specicity for PHL, because some
other diseases, such as congestive heart failure, Budd- Chiari
syndrome, or fatty liver, can also manifest hepatomegaly.
Diffuse type of PHL should be differentiated from these diseases. However, even normal liver images on CT or ultrasound cannot exclude hepatic involvement of lymphoma.
Therefore, the pathological examinations by core needle
biopsy in the rst choice for the differential diagnosis.
Nodular type of PHL should be differentiated from other
hepatic lesions, though no specic images can be used for
diagnosis of PHL. Marked hypo-echogenicity on US and
6.1.3 Dierential Diagnosis
penetrating signs on multiple imaging modalities may be
characteristic and specic imaging features for the diagnosis
PHL is a rare disease with non-specic clinical presentation,
varying laboratory and radiological features [3].
of hepatic lymphoma, and these need to be further
conrmed.
6 Rare Malignant Liver Tumors
147
• Hepatic involvements occurred secondary to systemic
lymphoma: Accompanying involvement of other organs,
especially spleen and generalized lymphadenopathy; the
involved liver shows diffuse inltration or multifocal
lesions.
• Hepatocellular carcinoma: Mostly correlated with cirrhosis; accompanied with elevated serum AFP; rich color
ow signal with high resistance index; homo-/heterogeneous arterial hyperenhancement followed with wash-out
in portal venous/late phase in contrast-enhanced imaging
modalities.
• Cholangiocarcinoma: Hypoechoic lesions with ill-dened
margin and distal intra-hepatic bile duct dilatation; frequently accompanied with elevated serum CA19-9; irregular peripheral rim-like hyperenhancement in arterial
phase followed quick wash-out in portal venous/late
phase on CEUS; delayed central enhancement in late
phase on contrast enhanced CT and MRI.
• Malignant liver metastasis: Frequently with a history of
primary cancer; mostly multiple lesions within the liver;
bull-eye sign in contrast enhanced imaging modalities,
that is thick rim-like enhancement in arterial phase followed with wash-out in portal venous/late phase.
• Hemangioma: Hypoechoic lesions with surrounding thin
hyperechoic circle with or without little color ow signal;
peripheral nodular enhancement with centripetal progression and no wash-out in contrast enhanced imaging
modalities.
DLBCL, BL, and B-LBL) are aggressive and can occur
over a wide age range, most small B-cell neoplasms (such
as CLL/SLL, low-grade FL, and MZL) occur mainly in
older patients and are indolent. Primary hepatic T-cell
lymphoma is exceedingly rare, representing only 5–10%
of PHLs, and usually follows an aggressive clinical
course. Nearly all cases are peripheral T-cell lymphoma
(PTCL), not otherwise specied (NOS), or hepatosplenic
T-cell lymphoma (HSTCL) [4].
• Pathological examination reveals either mass-forming or
inltrating growth patterns of tumor progression. The
mass-forming type shows an expansive and destructive
growth pattern, whereas the inltrating type shows inltration of tumor cells into the portal tracts as well as sinusoids. These pathological characteristics are signicantly
reected in the imaging studies of different modalities [5].
6.1.4.2 Staging, Grading, andClassication
The Ann Arbor criteria, similar to staging of lymphoma at
other sites, is used for the staging of PHL:
• Stage IE applies to disease localized within the liver.
• Stage IIE applies to the liver and regional lymph node/or
ipsilateral lymph node of the diaphragm.
• Stage IIIE applies to disease that involves the liver, the
lymph node basins on both sides of the diaphragm, and/or
the spleen.
• Stage IVE is used to describe disseminated disease.
Differential diagnosis can be difcult in some cases of
PHL with only imaging modalities, so pathological examination for a denitive diagnosis is still essential.
6.1.4 Pathology
6.1.4.1 General Features
• Primary hepatic lymphoma may be Hodgkin’s or Non-
Hodgkin’s, however, the latter is more common. The dis-
tinction between Hodgkin lymphoma (HL) and
non-Hodgkin lymphoma (NHL) is based on the patho-
logical ndings of Hodgkin and Reed-Sternberg cells
within abundant brogranulomatous background tissue in
HL.
• Most of PHLs are of B-cell type, and they include
DLBCL, Burkitt lymphoma (BL), B-lymphoblastic leu-
kemia/lymphoma (B-LBL), chronic lymphocytic leuke-
mia/small lymphocytic lymphoma (CLL/SLL), hairy cell
leukemia (HCL), follicular lymphoma (FL), mantle cell
lymphoma (MCL), and extranodal marginal zone lym-
phoma (MZL) of mucosa-associated lymphoid tissue
(MALT), with DLBCL being the most common subtype.
Although high-grade B-cell lymphomas (including
6.1.5 Clinical Issue
6.1.5.1 Presentation
• Commonly affecting middle-aged men, with a mean age
of 50–62 years (range: 21–86 years).
• Presenting non-specic symptoms (B-symptom) (fever,
night sweats, abdominal discomfort, and/or weight loss).
• Elevated lactate dehydrogenase, soluble interleukin-2
receptor (sIL-2R) may be predictive for disease
progression.
• Exact etiology is unclear, chronic viral infection (hepatitis
B virus, hepatitis C virus, and Epstein-Barr virus) and/or
immune dysfunction may cause PHL.
6.1.5.2 Prognosis
Although considered as an aggressive tumor, PHLs may
have a better prognosis than previously thought. However,
the prognosis varied among different histological subtypes, the prognosis of T-cell lymphoma is worse than that
of B-cell lymphoma. Furthermore, the differences of the
1- and 3-year survival rates between nodular PHL (70%
and 57%) and those of diffuse PHL (38% and 18%) were
signicant. Clinically, diagnosis challenges may be due to