Revision 31 for 'Giant hepatic venous malformation'

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Giant hepatic venous malformation

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

Terminology

It is important to note that according to newer nomenclature (ISSVA classification of vascular anomalies) 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. 

In addition, there is poor agreement in the literature as to the exact definition of what constitutes giant hepatic hemangiomas, 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 hemangiomas 3.

Clinical presentation

Many are asymptomatic, however hemorrhage, 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.

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

Pathology

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

Associations

Radiographic features

CT 

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

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

Typical enhancement pattern is of peripheral nodular enhancement that gradually fills centrally and follows aortic density (same as smaller hemangiomas).

Contrast filling may be slow and the central portions may never be demonstrated to fill in. Occasionally they may have no contrast enhancement.

Capsular retraction from scaring may also be seen.

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

Treatment and prognosis

Complications

Potential complications include: 

  • mass effect on adjacent structures (e.g. biliary tree, hepatic vessels)
  • Kasabach-Merritt syndrome 4 (form of consumptive coagulopathy due to thrombocytopaemia) 
  • rupture 

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