LIVER HEAMANGIOMAS

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LIVER HEAMANGIOMAS
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Definition Classification

• Hemangiomas are benign tumors of the endothelial
  cells which normally line the blood vessels.
• These tumors exhibit endothelial hyperplasia and
  form extra blood vessels.
• Approximately 60 of hemangiomas occur in the
  head and neck area. About 25 occur in the trunk
  and 15 occur in the arms or legs.
• Most (about 80) hemangiomas grow as a single
  tumor, while about 20 occur in multiple areas.
• While hemangiomas often grow within the skin,
  they can also develop in virtually any internal
  organ, including the liver, lungs,
  gastrointestinal tract, and even the brain.

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• Cavernous hemangioma is the most common primary
  liver tumor
• Its occurrence in the general population ranges
  from 0.4-20, as reported in an autopsy series
  (Karhunen, 1986).
• Hemangiomas are uncommon in cirrhotic livers the
  fibrotic process in cirrhotic liver may prohibit
  their development (Dodd, 1999).

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Pathophysiology

• The natural history of liver hemangioma is not
  completely understood.
• Hemangiomas are probably congenital in origin.
• Hereditary factors may play a role in the
  pathogenesis of some familial forms.
• Although the growth of hemangiomas is reported in
  the literature, ectasia is believed to contribute
  to lesion enlargement.
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  (Nghiem, 1997)
• Hemangiomas become fibrotic and shrink in
  patients with progressive cirrhosis (Brancatelli,
  2001).

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CONT..Pathophy..

• Several pharmacologic agents have been postulated
  to promote tumor growth.
• Steroid therapy, estrogen therapy, and pregnancy
  can increase the size of an already existing
  hemangioma.
• Hemangiomas also have been reported in pregnant
  women following ovarian stimulation therapy with
  clomiphene citrate and human chorionic
  gonadotropin.
• Whether any of these agents or conditions
  actually induces the development of hemangiomas
  de novo remains unclear.

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Clinical Presentation

• Sex female-to-male ratio of 51 to 61.
• Cavernous hemangioma of the liver affects both
  sexes equally in children and in autopsy series.
• Age Hemangiomas can occur in individuals of any
  age.
• They frequently occur in middle-aged women.
• Hepatic hemangiomas are rare in infancy.
• Have been detected prenatally in a growing fetus.

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Conti.. Clinical Present..

• The vast majority of hemangiomas (85) are
  asymptomatic.
• They most often are discovered when the liver is
  imaged for another reason or when the liver is
  examined at laparotomy or autopsy.
• Right upper quadrant pain is
• The most common complaint
• May result from thrombosis and infarction of the
  lesion, hemorrhage into the lesion, or
  compression of adjacent tissues or organs.
• Physical examination Infrequently,
• An enlarged liver.
• The presence of an arterial bruit over the right
  upper quadrant.

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Conti.. Clinical Present..

• Rarely, hemangiomas may present as a large
  abdominal mass.
• Other atypical presentations include
• Cardiac failure from massive arteriovenous
  shunting,
• Jaundice from compression of the bile ducts,
• Gastrointestinal bleeding from hemobilia, and
• Fever of unknown origin.
• An illness that resembles a systematic
  inflammatory process has been described with
  findings of fever, weight loss, anemia,
  thrombocytosis, increased fibrinogen level, and
  elevated erythrocyte sedimentation rate.

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Complications

• Complications depend on the size and location of
  the tumor.
• Pressure on the stomach and duodenum caused by
  large
• pedunculated lesions may cause vague
  abdominal pain, early
• satiety, nausea, and vomiting.
  (Tran-Minh, 1991).
• Pedunculated hemangiomas may twist and cause
  acute abdominal pain.
  
  (Tran-Minh, 1991)
• Compression of the inferior vena cava may result
  in Budd-Chiari syndrome.
  (Hanazaki,
  2001)
• Acute thrombosis may result in acute inflammatory
  changes that cause fever, abdominal pain, and
  abnormal liver function.
  
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  (Pol, 1998)
• Spontaneous or post-traumatic rupture is a
  catastrophic complication that occurs in about
  1-4 of hemangiomas it has a considerable
  mortality rate, as high as 60.
  (Cappellani, 2000)

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As Part Of Well-defined Clinical Syndromes

• In Klippel-Trenaunay-Weber syndrome, hepatic
  hemangiomas occur in association with congenital
  hemiatrophy and nevus flammeus, with or without
  hemimeganencephaly.
• In Kasabach-Merritt syndrome, giant hepatic
  hemangiomas are associated with thrombocytopenia
  and intravascular coagulation.
• Osler-Rendu-Weber disease is characterized by
  numerous small hemangiomas of the face, nares,
  lips, tongue, oral mucosa, gastrointestinal
  tract, and liver.
• Von Hippel-Lindau disease is marked by cerebellar
  and retinal angiomas, with lesions also in the
  liver and pancreas.
• Multiple hepatic hemangiomas have been reported
  in patients with systemic lupus erythematosus.
• Cutaneous hemangiomas are a common finding.
• No correlation was found between the presence of
  both cutaneous and hepatic hemangiomas in
  individual patients.

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Lab Studies

• Results usually are normal.
• Anemia and reduced hematocrit levels may be
  present in patients with ruptured hemangiomas.
• Thrombocytopenia can result from sequestration
  and destruction of platelets in large lesions.
• Hypofibrinogenemia has been attributed to
  intratumoral fibrinolysis.
• In patients with giant hemangiomas associated
  with Kasabach-Merritt syndrome, bleeding and
  clotting parameters may be abnormal.
• Normal alpha-fetoprotein and carcinogenic
  embryonic antigen (CEA) levels bolster clinical
  suspicion of a benign hepatic mass lesion.

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Differential diagnosis

• Hepatic hemangiomas should be differentiated from
  other hypervascular benign and malignant
  space-occupying liver lesions.
• Benign lesions include cysts, adenomas, focal
  nodular hyperplasia, and regenerating nodules.
• Malignant lesions include hepatocellular
  carcinoma and metastasis heamangioendothelioma.

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Pathology

• Usually, they occur as solitary lesions.
• They may be multiple in as many as 50 of
  patients (Mergo, 1998). No lobar predilection
  exists.
• Hemangiomas typically measure less than 5 cm
  some authors call those larger than 4-5 cm giant
  hemangiomas (Cappellani, 2000 Yang, 2001).
• Sizes range from 2 mm to more than 20 cm.
• Grossly, these lesions often appear as having a
  flat surface or as bulging subcapsular lesions.
• Lesions are reddish-blue and well demarcated from
  surrounding tissue.
• Large tumors may become pedunculated.
• They may be associated with focal nodular
  hyperplasia (Vilgrain, 2000).

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Histologic Findings

• Cavernous hemangioma are atypical or irregular in
  arrangement and size.
• Microscopically
• Mesenchymal in origin.
• Hemangiomas are composed of cavernous vascular
  channels lined by single layers of flattened
  endothelium and are separated by fibrous septa.
• These vascular spaces may contain thrombin,
  calcifications, or prominent scarring with
  hyalinization (sclerosed hemangioma).
• Malignant transformation has not been reported.

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• Most hemangiomas are incidentally detected at
  imaging studies.
• Ultrasonography is a cost-effective imaging
  modality for diagnosis of a hemangioma.
• CT and/or MRI may be required to specifically
  diagnose hemangioma

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Imaging Studies

• The modalities used for diagnosis of hepatic
  hemangiomas include ultrasonography (US),
  bolus-enhanced CT with sequential scans,
  single-photon emission computerized tomography
  (SPECT) with colloid 99m-labeled RBCs, magnetic
  resonance imaging (MRI), hepatic arteriography,
  and digital subtraction angiography (DSA).
• Ultrasound
• This is the most commonly initial diagnostic
  tool.
• Usually homogeneous
• Well-defined hyperechoic masses (though few can
  appear relatively hypoechoic when imaged within a
  fatty liver)
• Giant lesions can appear heterogeneous secondary
  to internal complex composition
• Posterior acoustic enhancement is commonly seen.
• CT scan
• Focal, well-circumscribed, low attenuation
  lesions on pre-contrast images
• Nodular, peripheral centripetal enhancement on
  dynamic contrast enhanced imaging 

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Gray-scale and Doppler sonograms show a
well-defined, uniformly hyperechoic liver mass
with peripheral feeder vessels. These features
are characteristic of a hemangioma
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Contrast-enhanced CT scan obtained during the
arterial-dominant phase demonstrates a hemangioma
with homogeneous and intense contrast enhancement
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Contrast-enhanced CT scans reveal the pathognomic
features of a hemangioma, namely, peripheral
nodular enhancement and progressive centripetal
fill-in (arrow). The smaller, peripheral lesion
(circled) shows homogeneous enhancement
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MRI

• Has a sensitivity and specificity of greater than
  90
• Can differentiate hemangiomas from other liver
  lesions especially the Small ones
• Typically hemangiomas are homogeneously
  hypointense relative to the liver on T1-weighted
  and markedly hyperintense (lightbulb sign) on
  T2-weighted images relative to the liver
• On dynamic, contrast-enhanced MR imaging,
  hemangiomas can demonstrate immediate homogeneous
  enhancement (lesions lt 1.5cm)
• Peripheral, nodular centripetal enhancement
  pattern progressing to homogeneity (lesions
  1.5-5cm)
• Peripheral nodular centripetal enhancement with
  persistent central hypointense region (lesionsgt
  5cm)

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• Giant cavernous hemangioma of the liver Axial
  T1-weighted pre-contrast imageshows a
  hypointense mass within the right hepatic lobe.
• Sequential enhanced delayed images
  showperipheral nodular centripetal enhancement
  with persistent central hypointensity

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Hemangioma appears as a hypointense mass on
T1-weighted MRIs and as a hyperintense mass on
dual-echo T2-weighted MRIs. Note that the signal
intensity of the lesion is similar to that of the
adjacent CSF
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Dynamic gadolinium-enhanced MRIs demonstrate the
progressive, centripetal contrast enhancement in
a hemangioma
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Single-photon emission computerized tomography
(SPECT)

• SPECT with colloid 99m-labeled RBCs appears to be
  as sensitive and specific as MRI.
• At present, SPECT scan is most likely the
  investigation of choice to confirm the diagnosis
  of hepatic hemangioma.
• The sensitivity for detecting large lesions of
  the liver (gt2-3cm) is also high. Hemangiomas as
  small as 0.5 cm may be detected with SPECT.

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SPECT images were obtained one hour post
injection. These demonstrate two foci of
increased blood pool activity the smaller one in
the left lateral lobe of the liver and a second
larger lobular one in the posterior right lobe of
the liver. These corresponded in location to the
hypodense lesions seen on the CT study
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SPECT examination Axial scans of blood-pool
scintigraphy with 99mTc-labeled erythrocytes A
well-circumscribed area (arrow) of increased
activity is present in the left lobe of the
liver, which indicates pathology with a high
blood content.
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Arteriography

• This invasive modality still may be useful in
  helping diagnose some hepatic hemangiomas.
• Branches of the hepatic artery may be displaced
  and crowded together or stretched around the
  lesion, with normal vascular tapering.
• Hemangiomas are characterized by the early
  opacification of irregular areas or lakes, with
  persistence of contrast in these areas long after
  arterial emptying. The hemangioma may appear as a
  ring or C-shaped lesion with an avascular center.
• Other Tests
• A precise diagnosis can be achieved in most cases
  by employing a combination of laparoscopy and
  fine-needle biopsy under sonographic guidance.

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• Liver biopsy
• Obtaining percutaneous biopsies of hepatic
  hemangiomas carries an increased risk of
  hemorrhage.
• It is contraindicated
• It may be reasonable in differentiation small
  liver lesion from hepatocellular carcinoma.
• It may shorten the diagnostic workup.

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Medical Care

• Most hepatic hemangiomas are small and
  asymptomatic at the time of diagnosis, and are
  likely to remain that way so they may be safely
  left alone.
• Routine follow-up
• Malignant transformation is not reported.
• Is not indicated in confirmed hepatic
  hemangiomas.
• Unless symptoms develop or tumor enlargement is
  suspected.
• Lesions should be reimaged every 3-6 months
• To rule out tumor enlargement in cases was in
  doubt at time of tumor detection.

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Management Of Large Hemangiomas

• No medical therapy is known to reduce the size or
  eliminate hepatic hemangiomas.
• The risk of rupture may warrant therapy in the
  case of large and symptomatic hepatic
  hemangiomas.
• Therapeutic modalities include
• surgical resection,
• surgical enucleation,
• arterial embolization,
• radiation therapy,
• orthotopic liver transplantation.
• Surgical resection is the treatment of choice.

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Arterial embolization

• Resection may not be possible due to
• Massive or diffuse nature of the lesion
• Its proximity to vascular structures.
• Branches of the hepatic artery can be embolized
  with polyvinyl alcohol and other substances.
• Embolization results in shrinking of the tumor,
  thereby minimizing the risk of complications.
• Complications Pain, fever, and nongranulomatous
  arteritis with eosinophilic infiltration.

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Hepatic irradiation and ligation of feeding
vessels

• Hepatic irradiation over 3 weeks has been
  reported to produce complete regression of
  hepatic hemangiomas, with minimal morbidity.
• Transhepatic compression sutures and selective
  ligation of large feeding vessels have
  successfully reduced intratumoral shunting that
  otherwise would have led to intractable cardiac
  failure.

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Surgical Treatment

• Surgery is indicated for
• symptomatic hemangiomas,
• rapidly growing tumors,
• Large lesions (gt10 cm).
• if hemangioma cannot be differentiated from
  hepatocellular carcinoma.
• Surgical resection
• An open or a laparoscope-assisted technique.
• Hepatic lobectomy may be necessary in the case of
  large lesions.
• The procedure is safe and well tolerated, with
  minimal postoperative morbidity and an average
  length of hospital stay of 6 days.
• In the absence of tumor-promoting factors such as
  estrogen therapy, tumors rarely recur after
  successful resection.

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Liver transplantation

• Patients with severe symptoms from large or
  diffuse hemangiomas that are not resectable can
  undergo total hepatectomy and orthotopic liver
  transplantation, with the expectation of good
  long-term results.

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• Consultations
• Consultation with a surgeon is warranted if
  resection is the choice of therapy.
• Diet
• No special dietary requirements or therapy is
  indicated.
• Activity
• No restriction of physical activity is indicated.
• Avoid trauma to right upper abdominal quadrant,
  especially if the hepatic hemangioma is large.
• No medications are useful to shrink or eradicate
  hepatic hemangiomas.

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Thank you

• By assistant lecturer
• Dina Ismail