Giant hepatic hemangioma

Changed by Matt A. Morgan, 6 Feb 2015

Updates to Article Attributes

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Giant hepatic venous malformations (also known as giant hepatic haemangiomas) are relatively uncommon non-neoplastic vascular lesions of the liver, which can be strikingly large and mimic tumours. 

Terminology

It is important to note that according to newer nomenclature (ISSVA classification of vascular anomalies) these, these lesions are merely known as slow flow venous malformations (ISSVA classification of vascular anomalies). Having said that, it is probably helpful in reports to include the word 'hemangioma' as this term is ubiquitous in the literature and mostmore familiar to many clinicians. The remainder of this article uses the terms 'giant hepatic hemangioma' and 'giant hepatic venous malformation' interchangeably. 

In addition, there is poor agreement in the literature as to the exact definition of what constitutes giant a "giant" hepatic haemangiomashaemangioma, as some of the literature defines as a size  > 4;4 cm but others >6 or even >10 cm. Either way, they are examples of atypical hepatic haemangiomas 3.

Clinical presentation

Many are asymptomatic, however haemorrhage, thrombosis, and mass effect may lead to abdominal fullness and pain. Depending on location and size compressivemass effects on adjacent structures (biliary dilatation or vascular structures) may also lead to presentation.

Haemorrhage and/or rupture (following trauma/biopsy typically) may also be encountered(typically following trauma or biopsy).

Pathology

Giant hepatic venous malformations may contains areas of central necrosis/liquefaction, haemorrhage, peripheral calcification, fibrosis and thrombosis, resulting in heterogenousheterogeneous appearance and incomplete opacification, even on very delayed imaging. 

Associations

Radiographic features

CT 

Giant haemangiomas may demonstrate similar findings to their smaller relatives, although findings are less consistent.

On non contrastnoncontrast scans the the lesions are usually heterogeneous hypo-attenuatinghypoattenuating masses with marked central areas of low attenuation.

TypicalThe typical enhancement pattern is of peripheral nodular discontinuous enhancement that gradually fills centrally and follows aortic densityattenuation (same as for smaller haemangiomas).

Contrast filling may be slow and the central portions may never be demonstrated todemonstrate complete contrast fill in. Occasionally, they may have nominimal contrast enhancement.

Capsular retraction from scaringscarring may also be seen.

MRI
  • T1
    • sharply marginated hypo intense, hypointense mass
    • cleft like-like areas of low signal intensity
  • T1 C+ (Gd)
    • cleft like-like area may remain hypo intensehypointense during enhancement
    • enhancement pattern is otherwise similar to that seen on CT
  • T2: cleft-like area may be markedly T2 hyper intensehyperintense

Treatment and prognosis

Complications

Potential complications include: 

  • mass effect on adjacent structures (e.g. biliary tree, hepatic vessels)
  • Kasabach-Merritt syndrome 4 (form(a form of consumptive coagulopathy due to thrombocytopaemiathrombocytopaenia
  • rupture with hematoperitoneum
  • -<p><strong>Giant hepatic venous malformations</strong> (also known as <strong>giant hepatic haemangiomas</strong>) are relatively uncommon non-neoplastic vascular lesions of the liver, which strikingly large and mimic tumours. </p><h4>Terminology</h4><p>It is important to note that according to newer nomenclature (<a href="/articles/issva-classification-of-vascular-anomalies">ISSVA classification of vascular anomalies</a>) these lesions are merely known as slow flow venous malformations. Having said that it is probably helpful in reports to include the word 'hemangioma' as this term is ubiquitous in the literature and most familiar to many clinicians. The remainder of this article uses the terms 'giant hepatic hemangioma' and 'giant hepatic venous malformation' interchangeably. </p><p>In addition, there is poor agreement in the literature as to the exact definition of what constitutes giant hepatic haemangiomas, as some of the literature defines as a size  &gt; 4 cm but others &gt;6 or even &gt;10 cm. Either way they are examples of atypical <a href="/articles/hepatic-haemangioma-3">hepatic haemangiomas</a> <sup>3</sup>.</p><h4>Clinical presentation</h4><p>Many are asymptomatic, however haemorrhage, thrombosis and mass effect may lead to abdominal fullness and pain. Depending on location and size compressive effects on adjacent structures (biliary dilatation or vascular structures) may also lead to presentation.</p><p>Haemorrhage and/or rupture (following trauma/biopsy typically) may also be encountered.</p><h4>Pathology</h4><p>Giant hepatic venous malformations may contains areas of central necrosis/liquefaction, haemorrhage, peripheral calcification, fibrosis and thrombosis, resulting in heterogenous appearance and incomplete opacification even on very delayed imaging. </p><h5>Associations</h5><ul><li><a href="/articles/hepatic-haemangiomatosis">hepatic haemangiomatosis</a></li></ul><h4>Radiographic features</h4><h5>CT </h5><p>Giant haemangiomas may demonstrate similar findings to their smaller relatives, although findings are less consistent.</p><p>On non contrast scans the lesions are usually heterogeneous hypo-attenuating masses with marked central areas of low attenuation.</p><p>Typical enhancement pattern is of peripheral nodular enhancement that gradually fills centrally and follows aortic density (same as smaller haemangiomas).</p><p>Contrast filling may be slow and the central portions may never be demonstrated to fill in. Occasionally they may have no contrast enhancement.</p><p>Capsular retraction from scaring may also be seen.</p><h5>MRI</h5><ul>
  • +<p><strong>Giant hepatic venous malformations</strong> (also known as <strong>giant hepatic haemangiomas</strong>) are relatively uncommon non-neoplastic vascular lesions of the liver, which can be strikingly large and mimic tumours. </p><h4>Terminology</h4><p>It is important to note that according to newer nomenclature, these lesions are merely known as slow flow venous malformations (<a href="/articles/issva-classification-of-vascular-anomalies">ISSVA classification of vascular anomalies</a>). Having said that, it is probably helpful in reports to include the word 'hemangioma' as this term is ubiquitous in the literature and more familiar to many clinicians. The remainder of this article uses the terms 'giant hepatic hemangioma' and 'giant hepatic venous malformation' interchangeably. </p><p>In addition, there is poor agreement in the literature as to the exact definition of what constitutes a "giant" hepatic haemangioma, as some of the literature defines as a size  &gt;4 cm but others &gt;6 or even &gt;10 cm. Either way, they are examples of atypical <a href="/articles/hepatic-haemangioma-3">hepatic haemangiomas</a> <sup>3</sup>.</p><h4>Clinical presentation</h4><p>Many are asymptomatic, however haemorrhage, thrombosis, and mass effect may lead to abdominal fullness and pain. Depending on location and size mass effects on adjacent structures (biliary dilatation or vascular structures) may also lead to presentation.</p><p>Haemorrhage and/or rupture <span style="line-height:20.7999992370605px">may also be encountered </span><span style="line-height:1.6">(</span><span style="line-height:20.7999992370605px">typically </span><span style="line-height:1.6">following trauma or biopsy).</span></p><h4>Pathology</h4><p>Giant hepatic venous malformations may contains areas of central necrosis/liquefaction, haemorrhage, peripheral calcification, fibrosis and thrombosis, resulting in heterogeneous appearance and incomplete opacification, even on very delayed imaging. </p><h5>Associations</h5><ul><li><a href="/articles/hepatic-haemangiomatosis">hepatic haemangiomatosis</a></li></ul><h4>Radiographic features</h4><h5>CT </h5><p>Giant haemangiomas may demonstrate similar findings to their smaller relatives, although findings are less consistent.</p><p>On noncontrast scans the lesions are usually heterogeneous hypoattenuating masses with marked central areas of low attenuation.</p><p>The typical enhancement pattern is peripheral nodular discontinuous enhancement that gradually fills centrally and follows aortic attenuation (same as for smaller haemangiomas).</p><p>Contrast filling may be slow and the central portions may never demonstrate complete contrast fill in. Occasionally, they may have minimal contrast enhancement.</p><p>Capsular retraction from scarring may also be seen.</p><h5>MRI</h5><ul>
  • -<li>sharply marginated hypo intense mass</li>
  • -<li>cleft like areas of low signal intensity</li>
  • +<li>sharply marginated, hypointense mass</li>
  • +<li>cleft-like areas of low signal intensity</li>
  • -<li>cleft like area may remain hypo intense during enhancement</li>
  • +<li>cleft-like area may remain hypointense during enhancement</li>
  • -<strong>T2:</strong> cleft area may be markedly T2 hyper intense</li>
  • +<strong>T2:</strong> cleft-like area may be markedly T2 hyperintense</li>
  • -<a href="/articles/kasabach-merritt-syndrome-1">Kasabach-Merritt syndrome</a> <sup>4</sup> (form of consumptive coagulopathy due to thrombocytopaemia) </li>
  • -<li>rupture </li>
  • +<a href="/articles/kasabach-merritt-syndrome-1">Kasabach-Merritt syndrome</a> <sup>4</sup> (a form of consumptive coagulopathy due to thrombocytopaenia) </li>
  • +<li>rupture with <a title="Hematoperitoneum" href="/articles/haemoperitoneum">hematoperitoneum</a>
  • +</li>

Tags changed:

  • liver
  • liver mri
  • slow flow vascular malformation

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