Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5762_Библиотеки_им_академика_М_И_Перельмана.pdf
168
e
Fig. 6.14 (continued)
Q. Lu et al.
f
6.4.2.6 Best Imaging Protocol Advices
• Conventional Ultrasound
– HBCAC should be suspected when a single unilocular
or multilocular multiseptated liver cystic or cysticsolid lesion is diagnosed.
– A nodule diameter >10 mm and the calcications
along the wall and internal septum on conventional
B mode ultrasound are suggestive of HBCAC.
– An extensive solid component and prominent mural
nodularity are suspected as HBCAC.
– Ultrasound is an ideal imaging method for detecting
septations and calcications that can be missed on CT
or MRI.
• Contrast Enhanced Ultrasound
– Contrast enhanced ultrasound can show the character-
istics of blood supply in cyst wall and internal septa of
HBCAC.
– Enhancements during arterial phase (10–30 s), portal
venous (30–120s), and late vascular phases (120–300s)
should be evaluated in liver cystic or cystic- solid lesion.
– A solitary complex cystic mass with internal septa,
mural or septal nodules, and papillary projections,
which are enhanced after intravenous contrast admin-
– T1-weighted images of HBCAC show low-signal in
the multilocular cystic part, with irregular compartments and soft tissue protrusions in the capsule.
– HBACA lesions are predominantly high in signal, and
intravascular compartments and solid protrusions
show low signal on T2-weighted images.
– With the difference in the presence of solid compo-
nents, protein content, and hemorrhage, the internal
uid within the HBCAC lesion can show varied signal
intensity on T1- and T2-weighted 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.
– MRCP images show hyperintense multilocular cystic
mass and possibly a ductal dilatation upstream to cystic lesion.
– The addition of diffusion-weighted MR aids in the
assessment of hepatic lesions with improved cyst characterization and detection of malignancy.
• The combination of ultrasound, CT, and MRI imaging
modalities can help diagnose HBCT with signicantly
improved diagnostic accuracy.
istration is suggestive of HBCAC.
– Contrast enhanced ultrasound is useful in depicting the
vascularity of the mural nodule and making a differen-
6.4.3 Dierential Diagnosis
tial diagnosis between a mural or septal nodule and
intra-cystic debris.
• CT
– Polypoid, pedunculated excrescences with signicant
contrast enhancement are more commonly seen in
HBCAC than in HBCA.
– CT can show tiny calcication of the internal
septations.
– Contrast enhanced CT scan can better show the
enhancement of cyst wall, internal septa, and septum
6.4.3.1 Hepatic Biliary Cystadenoma
• Hepatic biliary cystadenoma is a benign unilocular or
multilocular cystic primary tumor with multiple smooth,
thin internal septations, and absence of mural
nodularity.
• Rare manifestations of hepatic biliary cystadenoma
including papillary projections, internal septations with
nodular areas, mural nodules, and coarse calcications
along the wall are different from HBCA.
nodules.
• MRI
6 Rare Malignant Liver Tumors
169
6.4.3.2 Simple Hepatic Cysts
• It shows a smooth boundary and thin capsule wall and
lack of internal septations and papillary projections.
• In enhanced imaging, the capsule wall is no
enhancement.
• If the cyst is complicated by infection, the cyst wall can
also be seen to be thickened and strengthened, and the
density/signal of the cyst uid is uneven, which is difcult
to identify, and it needs to be comprehensively analyzed
in combination with clinical history and laboratory tests.
6.4.3.3 Hemorrhagic Hepatic Cysts
• Ultrasound can detect irregular intracystic clots and nodular septal images.
• It homogenously decreases spatial resolution on CT and
typically T1 bright on MR imaging.
6.4.3.4 Metastatic Tumor
• Most metastatic tumors have a clear history of the primary tumor.
• The lesion can be necrotic showing as a cystic tumor
without multilocular septa.
• The cyst wall is thick and irregular with ring enhancement, typically showing “bull’s eye sign.”
6.4.3.5 Hepatic Abscesses
• It has typical clinical symptoms, fever and other infection
symptoms.
• Ring enhancement in thick-wall, circular edema bands
around the focus, enhanced abscess walls, and peripheral
non-reinforced edema bands constitute “target ring sign”
can be seen on enhanced CT.
• Signicant increase in white blood cells is typical biochemical manifestation.
• The septations characteristic of cystadenomas are uncommon in abscesses.
6.4.3.6 Hydatid Disease
• It is typically caused by Echinococcus granulosus in animal husbandry area.
• Typical CT manifestations are multiocular cystic lesions
surrounded by daughter cysts.
• If the inner walls of the cysts rupture, it shows as “water
oating lotus” sign, part of the cysts may be accompanied
by arc-shaped calcication.
• If necessary, diagnosis will be combined with laboratory
tests.
6.4.3.7 Biliary Intraductal Papillary Mucinous
Neoplasm
• It occurs more commonly among Asians. The clinical and
imaging manifestations of the biliary intraductal papillary
mucinous neoplasm and HBCAC are very similar.
• The differential diagnosis mainly depends on pathological examination.
• Demonstration of cystic mass and intrahepatic bile duct
communication along with the presence of distal mucin is
the diagnostic clue of biliary intraductal papillary mucinous neoplasm.
• Most Biliary intraductal papillary mucinous neoplasms
have the dilatation of the bile ducts and are also prone to
gallstone.
6.4.3.8 Undierentiated Embryonal Sarcoma
• The tumor typically appears as a large, solitary, predominantly cystic mass with a well-dened border. And occasionally, a pseudocapsule could separate the mass from
normal hepatic parenchyma.
images and hyperintense on T2-weighted images on MRI
images.
• On contrast-enhanced CT and MRI images, heterogeneous enhancement is present in the solid (usually peripheral) portions of the mass, especially on delayed images.
• They have multiloculated cystic appearance but are generally seen in children and young adults.
6.4.3.9 Mesenchymal Hamartoma
• Mesenchymal hamartomas of the liver are uncommon
benign tumors.
• It is very similar to cystadenomas, but it is most often
seen in infants less than 2 years of age.
6.4.4 Pathology
6.4.4.1 General Features
• HBCAC is unilocular or multilocular septate cystic mass,
covered by brous wall of variable thickness, frequently
having cellular ovarian stroma in the cyst wall, having
nely granular or trabeculated inner surface or having
some polypoid masses projecting into the lumen, lined by
mucin-positive cuboidal or columnar epithelial cells.
• HBCAC is identied by the presence of proliferating
cytologically malignant epithelium. HBCAC tumor demonstrates disappeared cell polarity, inconsistent cell
nucleus size, nuclear pleiomorphism, cellular atypia, and
nuclear division.
• The presence of invasion, a loss of epithelial nuclear stratication, a tubule–papillary architecture and mild nuclear
pleomorphism, helps the diagnosis of a HBCAC.
• The uid within the cystic portion can be proteinaceous,
mucinous, and occasionally gelatinous, purulent, or hemorrhagic due to trauma.
• Intracellular uid composition of tumor cyst cavity can be
inconsistent.
170
Q. Lu et al.
6.4.4.2 Staging, Grading, andClassication
• There are two subsets of HBCAC pathologically (1) cystadenocarcinoma originating from a benign cystadenoma
with ovarian-like stroma between the inner epithelial lining and the outer connective tissue capsule (occurs exclusively in women) and (2) de novo cystadenocarcinoma
occurring almost exclusively in men.
• HBCAC is also divided into two growth types according
to clinicopathologic features: Noninvasive type (carcinoma cells conned to the cystic lesions) and invasive
type (carcinoma cells extending into the surrounding
hepatic parenchyma or neighboring organs).
6.4.5 Clinical Issues
6.4.5.1 Presentation
• Most common signs/symptoms
– Abdominal pain
– Asymptomatic
– Palpable mass, distension, nausea, vomiting, fever, and
occasionally biliary obstruction and jaundice
– Elevated CA199 level
6.4.5.2 Prognosis
• HBCACs have different origins. Most males originate
directly from the bile duct epithelium (without the presence of mesenchymal stroma, ovarian-like) and the tumor
progresses rapidly and the prognosis is poor. In females,
the malignant transformation of HBCA (with the presence of mesenchymal stroma, ovarian-like) results in
slow tumor progression and a good prognosis after complete tumor resection.
• The relatively low frequency (6%) of carcinomatous
changes in resected mucinous cystic neoplasm has been
reported.
• The recurrence rates of patients who have reached the
complete resection are lower than patients who have not
reached the complete resection.
• Radical resection and noninvasive tumor type are independent prognostic factors for the overall survival of
HBCT. The presence of spindle cell/ovarian stroma is a
risk of recurrence and worse outcomes.
6.5 Neuroendocrine Neoplasm
Wen-PingWang, Jia-YingCao, and YiDong
6.5.1 Terminology
Denitions
• NEN is a group of tumors originating from the neuroendocrine system, which are distributed throughout the
body, mainly occurring in gastrointestinal tract and pancreas. Neuroendocrine tumors are increasingly diagnosed,
either incidentally as part of screening results, or for
symptoms, which have commonly been mistaken for
other diseases initially. The World Health Organization
(WHO) 2010 nomenclature considers all NETs as malignant and classies them by the cellular proliferation and
differentiation degree.
• Since the blood supply of gastrointestinal tract and pancreas ow through portal vein, the liver is one of the most
frequently metastasized sites of NEN [36], and the primary neuroendocrine tumor arising in the liver is very
rare [37].
6.5.2 Image
6.5.2.1 Ultrasonographic Findings
PHNEN
• Most lesions show single hyperechoic mass in the liver on
conventional ultrasound and some lesions show mixed or
hypoechoic. Due to the high vascularity of the tumor,
color Doppler ow imaging usually detects arterial signals easily.
• When cystic degeneration happened, there was an irregular anechoic part inside.
• PHNEN is less likely to invade blood vessels, and so portal emboli are very rare.
MHNEN
6.4.5.3 Treatment
• Radical excision, either with a wide margin of normal
liver or by means of a typical lobectomy, depending on
the size and location of the lesion.
• Radiotherapy and chemotherapy are adjuvant therapy of
radical excision.
• Liver transplantation should be saved for selected cases
with complete hepatic involvement and poor hepatic
reserve where hepatectomy implies high risks.
• MHNEN has the characteristics of homogeneous echogenicity and less cystic part. Some lesions may have typical halos.
• It has been reported that the conventional ultrasound characteristics of MHNEN are related to the origin of the primary site, but the research of big samples is still not
enough.
• The blood ow detection rate of color Doppler ow imaging was lower than that of PHNEN.
a
6 Rare Malignant Liver Tumors
171
6.5.2.2 Contrast Enhanced Ultrasound Findings
PHNEN
• PHNENs were more likely to have an obvious hyperenhancement in arterial phase and hypoenhancement in portal venous and late phases after acoustic contrast agent
administration [38].
• The cystic part of the lesions was not enhanced in all
phases.
• The enhancement pattern was showed as “fast in and fast
out.”
MHNEN
• Three enhancement patterns were observed: “fast in and
fast out” (Figs.6.15, 6.16, 6.17, and 6.18), “synchronous in and fast out” (Fig.6.19) and “slow in and fast
out.”
• Three peak enhancement patterns of CEUS were
observed: homogeneous enhancement (Figs. 6.15 and
6.16), heterogeneous enhancement (Figs.6.17–6.19), and
rim-like enhancement (Fig.6.20).
• The onset time of wash-out of MHNEN maybe later than
that of other liver metastases, and the degree of wash-out
of MHNEN may be slighter than that of other liver
metastases.
• The enhancement patterns may be related to the degrees
of differentiation of HNEN, pathological levels of HNEN,
and previously treated or not.
6.5.2.3 CT Findings
PHNEN
• The lesions are hypodense and calcication is not commonly seen inner the lesions. Multi lesions are more seen
than single ones. The diameter of lesions is generally
b
c
Fig. 6.15 A case of metastatic hepatic neuroendocrine neoplasm. B
mode ultrasound showed multiple hyperechoic lesions in the liver (a).
Color ow signals can be detected around the tumors (b). The tumors
showed homogeneous hyperenhancement during the arterial phase (c)
and wash-out during portal venous and late phases (d, e). Dynamic
three-dimensional contrast enhanced ultrasound displayed the feeding
d
arteries of the lesions (f). On MRI, multiple hepatic lesions showed
hypointense signal on T1-weighted image (g) and hyperintense signal
on transverse T2-weighted image (h). After injection of contrast agent,
these lesions were demonstrated global hyperenhancement in arterial
phase (i) and punched wash-out during portal venous and late phases (j)
172
ij
e f
Q. Lu et al.
g
h
Fig. 6.15 (continued)
larger than 5cm and the density is less uniform. The primary tumor is prone to have internal bleeding, cystic area,
some lesions showed continuous heterogeneous enhancement [40].
or necrosis.
• After contrast agent administration, most of the solid
MHNEN
lesions were obviously enhanced in the arterial phase, and
a few present mild to moderate enhancement, peripheral
enhancement, and hypoenhancement [39]. The enhancement was decreased in portal phase and delayed phase and
• Low density with homogeneousness [41].
• The enhancement was obvious hyperenhancement in the
arterial phase after contrast agent administration. The
a
6 Rare Malignant Liver Tumors
173
appearance of portal phase of lesion was related to the
origin of primary lesions and pathological classication.
6.5.2.4 MR Findings
PHNEN
• Transverse T1-weighted images show hypointense lesions
and Transverse T2-weighted images show lesions with
slightly hyperintense signal. The signal became heterogeneous when cystic degeneration and necrosis happened [40].
• In arterial phase, the lesions show slightly hyperenhancement or rim-like enhancement. It was reported that
capsule- like enhancement is regarded as a thin rim-like
enhancement along the periphery of the lesion in portal
and delayed phases, and is histopathologically related to
brous capsules.
• All lesions appeared as marked hyperintensity on DWI
compared with surrounding liver parenchyma. This can
be explained by the small round cells with high nuclei–
cytoplasm ratio, and stromal brosis at histologic examination, which could lead to reduced diffusion ability of
intra- and extracellular water molecule.
MHNEN
• Cystic degeneration and necrosis were rarely observed in
MHNENs owing to their high vascularity and relatively
small size [42].
• Some researches revealed that intra-lesion uid–uid levels can be regarded as an indicator of MHNEN.
• The lesions usually have an obvious enhancement in arterial phase and reduced enhancement in portal venous and
late phases (Figs.6.15, 6.16, and 6.18).
6.5.2.5 Other Imaging Finding
• Since the Somatostatin Receptor is highly expressed on
the surface of NEN cells, NEN can be labeled with
Somatostatin analogues such as 111In-DTPA and 111 Inoctreotide (Somatostatin Receptor Scintigraphy, SRS).
• Relevant studies have shown that, compared with traditional imaging (US, CT, MRI), SRS is highly sensitive to
the detection of NEN primary lesion and intrahepatic
metastasis [43].
• SRS can also evaluate the relationship between mass and
adjacent organs and blood vessels, predicting the feasibil-
b
c
d
Fig. 6.16 A case of metastatic hepatic neuroendocrine neoplasm. A
hyperechoic metastatic hepatic neuroendocrine neoplasm (MHNEN)
lesion was detected near the diaphragm in the right hepatic lobe (a). No
color ow signals could be detected inside the lesion (b). On contrast
enhanced ultrasound, the lesion showed homogeneous hyperenhance-
ment during arterial phase (c) followed by wash-out in the portal venous
and late phases (d, e). The lesion showed homogeneity on both T1- and
T2-weighted images of MRI (f, g). On contrast-enhanced MRI, the
lesion showed rim-like hyperenhancement in arterial phase (h) and
hypoenhancement in portal venous phase (i)
174
e
Q. Lu et al.
f
g
h
i
Fig. 6.16 (continued)
a
e
6 Rare Malignant Liver Tumors
c
175
b
d
Fig. 6.17 A case of metastatic hepatic neuroendocrine neoplasm
(MHNEN). A slightly hyperechoic lesion with a small anechoic part
inside was observed on B mode ultrasound (a). Color ow signals could
be detected in the tumor and the RI was measured as 0.60 (b). The
tumor showed hyperenhancement during the arterial phase, and central
non-enhancement area could be detected (c). The lesion appeared
slightly hypoenhancement in portal venous and late phases (d, e)
176
a
Q. Lu et al.
ity of surgery. It may have a high value in the evaluation
of disease stage and prognosis.
6.5.2.6 Best Imaging Protocol Advices
• When a patient has primary NEN tumor and multiple
intrahepatic lesions with malignant manifestations in
imaging, the possibility of MHNEN should highly be
considered.
• The nal diagnosis still requires pathological evidence. In
addition to pathologic conrmation, the diagnosis of
PHNEN requires various imaging or endoscopic methods
to exclude the possibility of extrahepatic primary lesion.
A long-term follow-up is still needed.
6.5.3 Dierential Diagnosis
6.5.3.1 Hepatocellular Carcinoma
• HCC patients generally have a history of chronic liver diseases such as chronic hepatitis and cirrhosis. The serum
AFP is often elevated, and larger tumors are more likely
to have a portal vein invasion and tumor thrombus, while
HNEN patients usually don’t have such manifestations.
• Necrotic cystic degeneration often occurred in some
HNEN lesions. However, the occurrence of necrotic cystic degeneration in HCC was relatively rare.
• Decline time of HCC on CEUS was later than that of
HNEN, which may be helpful in differentiating the two
diseases.
6.5.3.2 Metastatic Hepatic Carcinoma
• Patients often have a history of primary tumor, and serum
CEA and CA-199 are often elevated.
• Since the blood supply of the tumor is relatively poor, the
decrease time on CEUS is earlier than MHNEN.
6.5.4 Pathology
6.5.4.1 General Features
• Macroscopically, PHNEN was larger than 3 cm in
diameter, and the tumors were round or irregular in
b
c
Fig. 6.18 A case of metastatic hepatic neuroendocrine neoplasm
(MHNEN). The lesion was isoechoic with an unclear margin on B
mode ultrasound (white arrow) (a). Color ow signals could be detected
around the lesion (b). The lesion showed branch-like hyperenhance-
d
ment in arterial phase (c, d). It showed rapid wash-out at 34s after
injection of contrast agent (e). The lesion was hypoenhanced in the portal venous and late phases (f, g)
e
6 Rare Malignant Liver Tumors
g
177
f
Fig. 6.18 (continued)
tary and often located in the right lobe of the liver. The
cut surface of the tumor is grey and brittle, and if there
is necrosis, it will appear as grey red fish flesh
(Fig.6.19).
• The diameter of MHNEN is less than 3cm, and it is more
common and distributed in bilateral liver lobes.
• In microscopy view, PHNEN is similar to
MHNEN.Moderately differentiated (G1, G2) tumor cells
are arranged into trabecular, alveolar, or gyrus formations. In poorly differentiated patients (G3), the tumor
shape with clear boundaries. PHNEN is mostly soli-
6.5.4.2 Staging, Grading, andClassication
• In 2010, WHO classied digestive system NEN into g1–
g3 pathological levels based on mitotic image and tumor
proliferation index (ki-67).
• The denition of each grade is as follows: (1) G1: mitotic
count, <2/10 high power elds (HPFs) and/or <2% Ki-67
index; (2) G2: mitotic count 2–20/10 HPFs and/or 3–20%
Ki-67 index; and (3) G3: mitotic count >20/10 HPFs and/
or >20% Ki-67 index.
• Related studies have shown that this grading system is also
valuable in evaluating the prognostic relevance of HNEN.
cells were arranged into large nests or parenchymal sheets
without organ-like structures. The cell nuclei showed
enlarged nucleto-plasm ratio.
6.5.5 Clinical Issues
• Under the view of electron microscopy, immunohistochemically labeled chromogranin A (CgA), synaptophysin (SYN), and neuro-specic enol (NSE) were often
positive.
6.5.5.1 Presentation
• There are two types of NEN based on endocrine function:
Functional NEN and nonfunctional NEN.The former can
produce corresponding clinical manifestations, such as