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210
F. Mao et al.
8.2.6.2 Amebiasis
– The conclusive diagnosis is made by the detection of
serum antibodies to the amoeba with a combination of image ndings.
8.2.6.3 Schistosomiasis
– Diagnosis is based on the nding of living eggs in stool
examination or positivity on serologic test.

8.3 Pathology

8.3.1 General features

8.3.1.1 Echinococcosis
E. granulosus (causes CE) [13] – Most common species – Solitary in single organ involvement, 70% in liver,
25% in lungs – Cyst enlarges 1–5cm per year, lled with clear uid – Often with daughter cysts, inside which may merge
internal septation – Three layers consist the wall of cyst under
microscopy
External layer is a peri-parasitic host tissue layer that encompasses the larval endocyst, inltrated with chronic inammatory cells, where variable calcication may occur. Laminated layer, the outer layer of the endocyst, is 1mm thick, avascular, eosinophilic, refractile, and chitinous; strongly PAS+, GMS+. Germinative layer, the innermost layer, gives rise to brood capsules that enlarge to form protoscolices, the separated capsules from the wall thus form a sediment called “hydatid sand.”
E. multilocularis (causes AE) [13] – Less common and slower growing than that of E.
granulosus. – Restricted to the cool region of Northern Hemisphere. – Liver is the most affected initial organ (99% of cases),
maybe followed by metastasis or invasion to other
organs. – Tissue invasion may simulate malignancy in each
focus. – Foci with alveolar structure consisted of numerous
irregular small cysts in 1–20mm inltrative into adja-
cent tissue, with calcication and necrosis.
– cyst with a thinner peri-cystic rim and laminated layer
compared to E. granulosus, usually with no germinal layer under microscopy.
8.3.1.2 Amebiasis
• Macroscopically, the hepatic amebic abscess is identied as a well-delineated area lled with necrotic tissue of yel­lowish or creamy consistency [11].
• A rind of congested hepatic tissue surrounds the abscess.
• The necrotic materials can be solid, semi-liquid, some­times containing mucus [11].
• The size of a hepatic abscess is variable, ranging from 5 to 15cm in diameter [11].
8.3.2 Staging, Grading, andClassication
8.3.2.1 Echinococcosis
• The World Health Organization Informal Working Group on Echinococcosis (WHO-IWGE) classication of Cystic Echinococcosis. In this classication, six cyst stages have been assigned to three clinical groups:
– The “active” group includes developing cysts, which
may be unilocular (CE1) or multivesicular with daughter vesicles (CE2) and which are usually found to be viable.
– The “transitional” group (CE3) includes both cysts
with detachment of endocyst (CE3a) and predomi­nantly solid cysts with daughter vesicles (CE3b).
– The “inactive” group (CE4 and CE5) exhibits involu-
tion and solidication of cyst content with increasing degrees of calcication and is nearly always found to be non-viable [9].

8.4 Clinical Issues

8.4.1 Presentation

8.4.1.1 Echinococcosis
• Clinical manifestation
– Asymptomatic for more than 10 years in slow-growing
cases [12].
– Depending on the size and location of lesion, epigas-
tric discomfort, chronic cough caused by mass effect are often observed. Late phase of AE often presents like malignant liver tumor causing liver dysfunction and metastasis [12].
8 Hepatic Parasitosis
211
– Complications caused by rupture of cysts include bili-
ary obstruction and bronchi stulae, bacterial infec­tion, and anaphylaxis [12].
• Laboratory – Hydatid uid is the major antigenic source for echino-
coccosis immunodiagnosis, lipoproteins antigen B (AgB), and antigen 5 widely used in serological assays for CE, AE serology is more reliable on E. multilocu- laris antigens (Em2 and Em492) [12].
8.4.1.2 Amebic Liver Abscess
• Clinical manifestation – Right upper abdomen pain, fever, and hepatomegaly
are the predominant symptoms of hepatic amebiasis [2].
– Jaundice is reported in cases with multiple lesions or a
very large abscess.
– Some important complications may occur as a result of
perforation. – Pulmonary abscesses. – Purulent pericarditis. – Subphrenic abscesses and peritonitis.
• Laboratory – A mild degree of anemia or leukocytosis is present. – Serological methods include enzyme-linked immuno-
sorbent assay (ELISA), indirect hemagglutination assay (IHA), and latex agglutination.
– The ELISA test for detection of the galactose-
inhibitable adherence protein of E. histolytica in serum and feces appears to be the most reliable and sensitive serology test.
8.4.1.3 Schistosomiasis
• Clinical manifestation – Acute schistosomiasis is seldom recognized [14]. – Right after exposure, patients frequently complain of
itching that is caused by skin penetration by larvae [14].
– Several weeks later, patients may complain of a variety
of symptoms including fever, chills, headache, hives, or diarrhea [14].
– With diminishing of acute symptoms, the condition
moves into chronic process, with liver cirrhosis and granulomatous inammation in the urinary tract and lungs [14].
– The most important complication is periportal or
Symmers pipestem brosis of the liver, resulting in splenomegaly and gastroesophageal varices [14].
• Laboratory – The denitive diagnosis for a schistosome infection is
made by microscopic detection of eggs in stool (for S.
mansoni and S. japonicum) or urine (for S. haemato­bium) samples [14].
– Antigen tests use two proteoglycans, circulating anodic
antigens (CAAs) and circulating cathodic antigens (CCAs) that can be detected in serum and in urine.
– Genomic tests targeting schistosome DNA sequences
enable a species-specic diagnosis [14].

8.4.2 Prognosis

8.4.2.1 Echinococcosis
• Curative treatment is achieved by the complete removal
of the cyst, CE has a better prognosis than AE [12].
• Because of the complications, prognosis ranges widely [12].
• While in general, the earlier the treatment, the lower the
grade of the mass, the less the number, and the smaller the volume, the better the prognosis [12].
8.4.2.2 Amebiasis
• Amebicidal drugs are effective therapy.
• The time for termination of therapy varies from 1.5 to 23
months (median, 7 months).
• A residual hepatic cysts or focal regions may exist in
curative cases.
8.4.2.3 Schistosomiasis
• Curative rates of Praziquantel (PZQ) reach to 60–90% [14].
• While, re-infection is also a problem in endemic regions [14].
• The complication resulted from liver cirrhosis and urinary
bladder granulomatous inammation may affect the long­term prognosis of patients [14].

8.4.3 Treatment

8.4.3.1 Echinococcosis
• For CE, an image-based, stage-specic approach is help-
ful for choosing one of the following options: (1) percuta­neous treatment, (2) surgery, (3) anti-infective drug treatment, or (4) watch and wait [12].
212
F. Mao et al.
• For AE, early diagnosis and radical surgery remain one of the key elements. However, most patients with AE are diagnosed at a later stage, thus the backbone of AE treat­ment remains the continuous medical treatment [12].
8.4.3.2 Amebiasis
• The treatment of hepatic amebiasis is made by medical therapy, with metronidazole as the initial drug, followed by a luminal amebicide.
• In patients with large abscesses, and especially those with no response to medical treatment, percutaneous drainage must be performed under ultrasound or computerized tomography guidance.
• Surgical drainage by laparotomy is reserved for patients with secondary infections.
8.4.3.3 Schistosomiasis
• WHO recommends that PZQ be used at a single dose of 40mg/kg [14].
• Multiple features have made PZQ the drug of choice for treating schistosomiasis, including [14]:
– It is effective against all the three major species, S.
mansoni, S. haematobium, and S. japonicum.
– It can be given as a single oral dose and is usually well
tolerated.
– Its relatively low cost.

References

3. Pedrosa I, Saíz A, Arrazola J, Ferreirós J, Pedrosa CS. Hydatid disease: radiologic and pathologic features and complications. Radiographics. 2000;20:795–817.
4. Manzella A, Ohtomo K, Monzawa S, Lim JH.Schistosomiasis of the liver. Abdom Imaging. 2008;33:144–50.
5. Richter J, Botelho MC, Holtfreter MC, Akpata R, El Scheich T, Neumayr A, Brunetti E, etal. Ultrasound assessment of schistoso­miasis. Z Gastroenterol. 2016;54:653–60.
6. Li J, Dong J, Yang L, Li X, Song T.Comparison of [18-F]uorode­oxyglucose positron emission tomography and contrast-enhanced ultrasound for evaluation of hepatic alveolar echinococcosis activ­ity. Ultrasound Med Biol. 2018;44:2199–208.
7. Reinehr M, Micheloud C, Grimm F, Kronenberg PA, Grimm J, Beck A, Nell J, etal. Pathology of echinococcosis: a morphologic and immunohistochemical study on 138 specimens with focus on the differential diagnosis between cystic and alveolar echinococ­cosis. Am J Surg Pathol. 2020;44:43–54.
8. Kern P, Wen H, Sato N, Vuitton DA, Gruener B, Shao Y, Delabrousse E, etal. WHO classication of alveolar echinococcosis: principles and application. Parasitol Int. 2006;55(Suppl):S283–7.
9. Brunetti E, Tamarozzi F, Macpherson C, Filice C, Piontek MS, Kabaalioglu A, Dong Y, etal. Ultrasound and cystic echinococco­sis. Ultrasound Int Open. 2018;4:E70–e78.
10. Kodama Y, Fujita N, Shimizu T, Endo H, Nambu T, Sato N, Todo S, etal. Alveolar echinococcosis: MR ndings in the liver. Radiology. 2003;228:172–7.
11. Brunetti E, Kern P, Vuitton DA.Expert consensus for the diagno­sis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop. 2010;114:1–16.
12. Kantarci M, Bayraktutan U, Karabulut N, Aydinli B, Ogul H, Yuce I, Calik M, etal. Alveolar echinococcosis: spectrum of ndings at cross-sectional imaging. Radiographics. 2012;32:2053–70.
13. Srinivas MR, Deepashri B, Lakshmeesha MT.Imaging spectrum of hydatid disease: usual and unusual locations. Pol J Radiol. 2016;81:190–205.
14. Salles JM, Moraes LA, Salles MC.Hepatic amebiasis. Braz J Infect Dis. 2003;7:96–110.
1. LoVerde PT.Schistosomiasis. Adv Exp Med Biol. 2019;1154:45–70.
2. Kimura K, Stoopen M, Reeder MM, Moncada R.Amebiasis: mod­ern diagnostic imaging with pathological and clinical correlation. Semin Roentgenol. 1997;32:250–75.

Hepatic Inflammatory Pseudotumor

YiDong, Pei-LiFan, andWen-PingWang
9

9.1 Terminology

• Inammatory pseudotumor of the liver (IPT) is a rare
benign lesion composed of brous tissue, lymphocytes, histiocytes, and plasma cells.
• It was once described as an inammatory myobroblastic
tumor, histiocytoma, plasma cell granuloma, and broxanthoma.
• Without specic clinical and imaging features, IPT is
often misdiagnosed as hepatic malignant tumors [1].

9.2 Imaging

9.2.1 Conventional Ultrasound Findings

• Conventional B mode ultrasound manifestation of IPT
varies in different lesions.
• Generally, most lesions show a heterogeneous hypoechoic
mass with an irregular shape, without acoustic enhance­ment behind (Fig.9.1).
• The common and specic imaging of IPT shows a well-
circumscribed hypoechoic mass with a dumbbell shape (Fig.9.2).
• Color ow imaging can only detect blood signals at the
inammatory zone, because necrosis occurs inside the lesions frequently (Fig.9.3).
• There are several types of IPT on contrast enhanced ultra­sound (CEUS), including diffuse homogeneous hyperen­hancement (Figs. 9.1 and 9.4), diffuse heterogeneous hyperenhancement (Fig. 9.3), peripheral rim-like enhancement, and diffuse isoenhancement (Fig. 9.5) in arterial phase.
• Most IPT nodules show hypoenhancement during portal venous and late phases (Figs.9.3, 9.4, 9.5, 9.6, and 9.7).
• Nonenhancement of the entire lesion at all phases is spe­cic, which makes it easy to diagnose coagulative necro­sis inside the lesions (Fig.9.8). Sometimes, a slim rim-like hyperenhancement surrounding the lesion can be observed in the arterial phase (Fig.9.2). Nowadays, such lesion is called solitary necrotic nodule of the liver, pathologically characterized by central necrotic core enclosed by a hya­linized brotic tissue containing elastic bers with inam­matory cells.
• When it comes to the wash-in and wash-out patterns, some IPT lesions with arterial hyperenhancement show rapid wash-in and wash-out, while some IPT lesions show quickly wash-in and slowly wash-out (Fig.9.9) [2].
• Centrifugal wash-out is of high diagnostic ability in dif­ferentiating IPTs from hepatocellular carcinomas (Figs.9.1 and 9.6).

9.2.3 CT Findings

• IPT can manifest various patterns on images according to

9.2.2 Contrast Enhanced Ultrasound Findings

• Different lesions have different presentation enhancement patterns due to different ratios of histological components at different processes of inammatory.
Y. Dong (*) · P.-L. Fan · W.-P. Wang Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China e-mail: dong.yi@zs-hospital.sh.cn; fan.peili@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_9
different degrees of brosis and cellular inltration.
• Most tumors show low density on unenhanced CT, and heterogeneously enhancement or peripheral delayed enhancement in the arterial phase [3].
213
214
a
Y. Dong et al.
b
c
e
d
f
Fig. 9.1 A case of inammatory pseudotumor of the liver (IPT). Multiple irregular heterogeneous hypoechoic lesions were detected both in right lobe (a) and in left lobe (b) of liver on B mode ultrasound. Dotted color ow signals could be detected inside the lesions (c). Arterial Doppler spectrum with moderate resistance index (RI) as 0.64
was measured (d). The lesion showed homogeneous hyperenhancement with a vague margin in arterial phase (e, f) followed by centrifugal wash-out in portal and late phases (g, h, i) (arrow). This case was con­rmed by surgery and histopathological results (j)
9 Hepatic Inammatory Pseudotumor
215
g
h
ji
Fig. 9.1 (continued)

9.2.4 MRI Findings

• On MRI, the lesions are hypointense on T1-weighted images and isointense or heterogeneous hyperintense on T2-weighted images.
• Peripheral enhancement without central enhancement is a specic enhancement pattern of IPTs.
• During dynamic contrast enhanced MRI, most lesions progressively or persistently enhance.
• The lesions show diffuse homogeneous or heterogeneous hyperenhancement with delayed capsule-like enhance­ment without central enhancement.
• On DWI, all lesions are hyperintense in various degrees.

9.2.5 Other Imaging Findings

• FDG-PET sometimes confuses IPT with liver malignant tumors.
• Some studies have reported that IPT shows high dose uptake of FDG mimicking HCC lesions [4]. FDG uptake can be observed in various diseases, not specic to liver malignancies.
• IPT could show various manifestations on FDG-PET.

9.2.6 Best Imaging Protocol Advices

• The history and imaging manifestation of IPT is nonspecic.
• In clinical practice, IPT cannot be discriminated from malignant tumors.
• The following features are useful to make a diagnosis for IPT.
– No enhancement of the entire tumor is observed. – The IPT lesion has an irregular shape or a dumbbell
shape.
216
a
e
Y. Dong et al.
b
c
d
f
Fig. 9.2 A case of inammatory pseudotumor of the liver (IPT). A hypoechoic lesion with dumbbell shape was detected in the right lobe of liver (a). No ow signal could be detected in the lesion (b). The lesion showed slim rim-like hyperenhancement surrounding the lesion
in the arterial phase (c), suggesting a hyalinized brotic tissue contain­ing elastic bers with inammatory cells. The lesion showed non­enhancement at all phases (d, e, f), and the dumbbell shape of the lesion was extremely clear in the late phase (f)
a
f
e
9 Hepatic Inammatory Pseudotumor
217
b
c
d
Fig. 9.3 A case of heterogeneous hypoechoic inammatory pseudotu­mor (IPT) lesion was detected on B mode ultrasound (a). Dotted color ow signals could be detected in the periphery of the lesion (b). The lesion showed heterogeneous hyperenhancement in arterial phase (c),
with multiple non-enhanced areas inside (d). During portal venous phase, the lesion showed rapid wash-out and manifested hypoenhance­ment (e, f)
218
e
Y. Dong et al.
a
c
b
d
f
g
h
Fig. 9.4 A case of inammatory pseudotumor of the liver (IPT). A hypoechoic heterogeneous focal liver lesion with a vague margin was detected in the right lobe of liver (a). Dotted color ow signals could be detected in the peripheral area of the lesion (b). The lesion showed het­erogeneous hyperenhancement in arterial phase (c). When reaching the
peak enhancement, the enhanced area of lesion was larger than that on B mode ultrasound (d, e). The lesion showed early wash-out at 27s injection of contrast agents (f). During portal venous phase (g) and late phase (h), the lesion showed hypoenhancement
a
e
9 Hepatic Inammatory Pseudotumor
219
b
c
d
f
Fig. 9.5 A case of heterogeneous hypoechoic inammatory pseudotu­mor of the liver (IPT) lesion with a vague margin and an irregular shape was detected in the left lobe of liver (arrow) (a). The lesion showed
isoenhancement in the arterial phase (b, c) followed by wash-out in the portal venous and late phase (arrow) (e, f). The shape and margin of the lesion was much clearer in the late phase (arrow) (f)