Giant hepatic hemangioma
Updates to Article Attributes
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, these lesions are merely known as slow flow venous malformations (ISSVA classification of vascular anomalies). Having said that, it is probably helpful to include the word 'haemangioma' in reports, 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.
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 >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, mass effect on adjacent structures (biliary tree or vascular structures) may also lead to specific clinical presentations.
Haemorrhage and/or rupture may also be encountered (typically following trauma or biopsy).
Pathology
Giant hepatic venous malformations may contain areas of central necrosis/liquefaction, haemorrhage, peripheral calcification, fibrosis, and thrombosis, resulting in heterogeneous appearance and incomplete enhancement, 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-contrast scans, the lesions are usually heterogeneously hypoattenuating masses with marked central areas of low attenuation.
The typical enhancement pattern is peripheral nodular discontinuous enhancement that gradually fills centrally and follows aortic attenuation (same as for smaller haemangiomas).
Contrast filling may be slow and the central portions may never demonstrate complete contrast fill in. Occasionally, they may exhibit minimal contrast enhancement.
Capsular retraction from scarring may also be seen.
MRI
-
T1
- sharply marginated, hypointense mass
- cleft-like areas of low signal intensity
-
T1 C+ (Gd)
- cleft-like area may remain hypointense during enhancement
- enhancement pattern is otherwise similar to that seen on CT
- T2: cleft-like area may be markedly T2 hyperintense
Treatment and prognosis
Complications
Potential complications include:
- mass effect on adjacent structures (e.g. biliary tree, hepatic vessels)
-
Kasabach-Merritt syndrome 4 (a form of consumptive coagulopathy due to
thrombocytopaeniathrombocytopenia) - rupture with
haematoperitoneumhaemoperitoneum
-<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 to include the word 'haemangioma' in reports, 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 >4 cm but others >6 or even >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 effect on adjacent structures (biliary tree or vascular structures) may also lead to specific clinical presentations.</p><p>Haemorrhage and/or rupture may also be encountered (typically following trauma or biopsy).</p><h4>Pathology</h4><p>Giant hepatic venous malformations may contain areas of central necrosis/liquefaction, haemorrhage, peripheral calcification, fibrosis, and thrombosis, resulting in heterogeneous appearance and incomplete enhancement, 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 heterogeneously 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 exhibit minimal contrast enhancement.</p><p><a title="Hepatic capsular retraction" href="/articles/hepatic-capsular-retraction">Capsular retraction</a> from scarring 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 to include the word 'haemangioma' in reports, 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 >4 cm but others >6 or even >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 effect on adjacent structures (biliary tree or vascular structures) may also lead to specific clinical presentations.</p><p>Haemorrhage and/or rupture may also be encountered (typically following trauma or biopsy).</p><h4>Pathology</h4><p>Giant hepatic venous malformations may contain areas of central necrosis/liquefaction, haemorrhage, peripheral calcification, fibrosis, and thrombosis, resulting in heterogeneous appearance and incomplete enhancement, 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 heterogeneously 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 exhibit minimal contrast enhancement.</p><p><a href="/articles/hepatic-capsular-retraction">Capsular retraction</a> from scarring may also be seen.</p><h5>MRI</h5><ul>
-<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 href="/articles/haemoperitoneum">haematoperitoneum</a>- +<a href="/articles/kasabach-merritt-syndrome-1">Kasabach-Merritt syndrome</a> <sup>4</sup> (a form of consumptive coagulopathy due to thrombocytopenia)</li>
- +<li>rupture with <a href="/articles/haemoperitoneum">haemoperitoneum</a>