3. Hemangioma: epidemiology
• Hemangiomas are the most common benign
mesenchymal tumors of the liver, with a
prevalence range of 3-20%
• They are thought to arise from congenital
hamartomas that increase in size because of
progressive ectasia or from dilatation of existing
blood vessels in normally developed tissue
• Size: range from a few mm to > 20cm
• Age: all age groups, but are most commonly
discovered between third and fifth decades of life
4. Hemangioma: gender
• Gender: Female/male: 3:1; tend to be larger and
more numerous in women
• Hormonal influence is controversial
– It may grow in size during pregnancy and in women
taking oral contraceptives (OCP)
– Estrogen receptors have been identified on some
hemangioma
– Estrogen treatment in vitro can cause proliferation of
hemangioma vascular endothelial cells
– Case-controlled study found no causal link between
OCP use and hemangioma development
– Hemangioma growth does occur in the absence of
OCP use and in postmenopausal women or men
5. Hemangioma: clinical manifestations
• Often are small and asymptomatic; detected as an
incident mass on imaging
• Some may present with RUQ abdominal pain, fullness,
nausea, vomiting, easy satiety
• Pain may secondary to infarction, hemorrhage, torsion
or distension of the Glissen’s capsule
• Hepatic biochemical tests are usually normal except in
complications such as thrombosis, bleeding or biliary
obstruction
• Hemobilia may occur when bleeding into the biliary
tree
• Kasabach-Merritt syndrome: consumptive
coagulopathy causing DIC
8. Adenoma: epidemiology (I)
• Hepatic adenoma are benign epithelial liver tumors, seen
most commonly in women of childbearing age
• It tends to develop in individuals with a hormonal or
metabolic abnormality that can stimulate hepatocyte
porliferation
• The number of reported cases of hepatic adenomas has
increased dramatically since 1960s, coinciding with the
introduction of oral contraceptives (OCP)
• Annual incidence: 1 per million (no OC women)/30-40 per
million (long-term users)
• Usually larger in size, more numerous and more likely to
bleed in patients taking OCP
• It may regress with cession of OCP and increase in size
during pregnancy.
9. Adenoma: epidemiology (II)
• Adenomas are also seen in association with
glycogen storage diseases
• Incidence: 50% in type Ia; 25% in type III
• Usually occur more commonly in males before
the third decade of life
10. Adenoma: clinical manifestations
• Often asymptomatic and noted incidentally on imaging
studies
• Up to 25% of patients present with abdominal pain in
the epigastrum or RUQ abdomen
• Acute or severe abdominal pain maybe caused by
bleeding, rupture into peritoneum or tumor necrosis
• The risk of bleeding increased with
– Pregnancy
– Prolonged OCP
– Multiple adenoma
– Larger lesions
– Sub-capsular location
11. Subtypes of hepatocellular adenoma:
more than a uniform benign tumor
• Hepatocyte nuclear factor-1ɑ (HNF1ɑ)
inactivating mutations: 36-46%
• Inflammatory subtype (IHCA): 18%-44%
• β-catenin: 13%-14%
– 5%: β-catenin activating mutations
– 7%: β-catenin mutations with IHCA features
• Unclassified: 9%-23%
16. Clinical and genetic determinants of occurrence of
hepatic adenoma
Nault JC, et al. Gastroenterology 2013; 144:888-902
17. Adenoma: diagnosis
• Hepatic biochemical tests are usually normal
– Alk-P and GGT may occasionally be
elevated, particularly in patients with bleeding or
multiple adenomas
– AFP is usually normal
• Percutaneous liver biopsy is usually not indicated
(?)
– Risk of bleeding
– Lesions may have loci of malignancy that may be
missed on biopsy
– Often insufficient in providing the necessary tissue for
histological diagnosis
20. FNH: epidemiology
• FNH accounts for 25 % of benign hepatic
tumor and is second in frequency only to
hemangioma.
• It is more common in females (female/male
ratio: 8/1).
• Roughly 20% of all cases are characterized by
multiple tumors.
• Most of the lesions are identified between the
third and fourth decades of life.
21. FNH: clinical manifestations
• In the vast majority of cases, FNH is initially asymptomatic
• No malignant transformation tendency in the follow-up
• Hemorrhage and necrosis are rare (2-3%)
• Oral contraceptives are not implicated, however, the risk of
hemorrhage appears to be higher in women taking oral
contraceptives
• Typically, the lesion is a solid tumor, often in the right lobe, with
a fibrous core and stellate projections
• Technetium scan usually shows a hot spot because of the
presence of Kupffer cells. Vascular on angiography, and septations
may be detectable by angiography, helical CT scan, and, most
reliably, MRI, but only rarely by sonography.
• OP is indicated only for symptomatic lesions
27. Hepatic Angiomyolipoma (AML)
Lu HC, Chau GY, Su CW. Hepatic
angiomyolipoma mimicking
hepatocellular carcinoma.
Gastroenterology 2009;136:1169
28. Hepatic Angiomyolipoma (AML)
• A rare mesenchymal tumor of the liver, which is
composed of a varying heterogeneous mixture of
adipose tissue, proliferating blood vessels, and smooth
muscle cells.
• Image manifestations of hepatic AML differ widely due
to the various proportions and distribution of the three
components, which make it difficult to establish an
accurate diagnosis. It is easily misdiagnosed as HCC
before surgery.
• The preoperative diagnostic accuracy was lesser than
32 % with the combined appliances of imaging survey
and fine-needle aspiration biopsy.
31. Hepatic epithelioid
hemangioendothelioma (HEHE)
• A very rare vascular neoplasm of the liver that arises from
endothelial cells, with an estimated incidence of 1 in 1,000,000 in
the whole population
• A borderline neoplasm at the middle of the spectrum, between the
benign epithelioid hemangioma and highly aggressive epithelioid
angiosarcoma
• Immunohistochemical identification of epithelioid and positive
dendritic cells for endothelial markers (Factor VIII-related antigen,
CD34, CD31) is essential for diagnosis
• Age: 12-86 years (mean: 41.7 years)
• Gender: female predominant (61%)
• May be associated with OCP, exposure to vinyl chloride, or major
hepatic trauma
• Clinical course: variable and unpredictable
32. Symptoms and signs of hepatic
epithelioid hemangioendothelioma
Mehrabi A, et al. Cancer 2006; 107:2108-21
33. Clinical and laboratory findings of hepatic
epitheloid hemangioendothelioma in Taiwan
Hsieh MS, Shun CT, et al. J Formos med Assoc 2010;109:219-27
34. Hepatic angiosarcoma
• Primary hepatic angiosarcoma accounting for
onely 0.1-2% of all malignant primary liver
tumors
• Highly malignant and rapidly progressive
vascular tumor of endothelial cell origin
• Environmental carcinogens: vinyl chloride,
thorium dioxide, arsenic
• Symptoms: abdominal pain, weakness, fever,
and weight loss
37. Hepatic TB: clinical manifestation
• Hepatic TB was rare in immunocompetent
patients
• Bolukbas and colleagues reported that only 4 of
88 (4.5%) abdominal TB patients had hepatic
involvement
• It had diverse and non-specific clinical
symptoms and signs including low grade
fever, night sweat, cough, abdominal
pain, hepatomegaly, body weight
loss, fatigue, vomiting, diarrhea, dyspnea, and
jaundice
38. Hepatic TB: clinical manifestation
• There are three types of hepatic TB
– The most common form is the miliary tuberculosis of
liver associated with lung TB, with diffuse fine
miliary micronodular lesions (less than 2 cm in
diameter on CT scan) in the liver parenchyma.
– The second type is the granulomatous disease or
tuberculous hepatitis, presenting with jaundice or
hepatomegaly, which is caused by caseating
granulomas in the liver.
– The least frequent type is the macronodular form
(also named pseudotumor or
tuberculoma), characterized by large (more than 2
cm) nodular lesions.
39. Hepatic TB
Tai WC, Chuah SK, Lee CM, et al. J Intern Med Taiwan 2008;19:410-7
40. Hepatic TB
• Differential diagnosis
– Abscess: early in the evolution of the lesion, its
appearance on contrast-enhanced images is similar to
abscess, whereas more advanced lesions usually calcify
– Metastases
– Hepatocellular carcinoma
– Masses with calcification: epitheloid
hemangioendothelioma; fibrolamellar carcinoma
– The finding of caseating granuloma in the liver biopsy
is considered diagnostic of TB
43. Cystadenoma: epidemiology
• Occur more commonly in women, range in
size from 2cm to 28 cm and can occur at
extremes of age, although usually present in
the fourth decade of life
• No known association between cystadenomas
and use of OCP
44. Cystadenoma: clinical manifestation
• Most commonly found incidentally on abdominal
imaging
• May present with abdominal pain and anorexia
• DDx: simple cyst
– Sonography: Hypoechoic lesions with irregular walls
and septations
– Histological examination:
• Contain blood or dark brown material
• Microscopic: lined by mucus-secreting cuboidal or columnar
epithelium, supported by cellular fibrosis stroma.
45. Polycystic liver disease
• Secondary to a failure in intralobular bile duct
involution during fetal development; the
intralobular ducts subsequently become
distorted and degenerate into cysts
• Usually diagnosed in the fourth and fifth
decades of life when symptoms develop
• Frequently associated with autosomal
dominant polycystic kidney disease
46. Polycystic liver disease: clinical
manifestations
• Abdominal discomfort, distention, nausea, and
vomiting
• Rupture or bleeding into a cyst may be associated
with severe acute abdominal pain
• Hepatic complications:
– Infection, bleeding, rupture, portal
hypertension, biliary obstruction , cholangiocarcinoma
(rarely)
• Hepatic biochemical tests: usually normal;
obstructive jaundice, ascites
• High-resolution brain CT scan for screening
intracranial aneurysms
