Cavernous Liver Haemangioma

DEFINITION
Benign endothelial cell tumours of the liver
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EPIDEMIOLOGY

Incidence

Most common benign liver tumours.
Age
Middle age most commonly
Gender
5F:1M

Risk factors
?oestrogen-driven

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AETIOLOGY

Benign tumours.
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BIOLOGICAL BEHAVIOUR

Pathophysiology

Endothelial cell tumours
- from endothelial cells that differ from sinusoidal cells in phenotype and function.
- build multiple vascular channels
- gain blood supply mainly from hepatic a.
--> important point for inflow control.
Vascular ectasia rather than hyperplasia or hypertrophy.
Pain usually relates to:
- stretching of Glisson's capsule;
--> stimulates nociceptive afferent nerve fibres; visceral pain that is difficult to describe.
- or development of thrombosis within.

Pathology
Usually intraparenchymal
- sometimes pedunculated.
Vascular lesions, blood-rich within endothelium and collagen.
- but often occluded, by thrombi due to prothrombotic conditions
- and because of abnormal blood flow through these irregularly-shaped vessels.
- endothelial markers CD31, CD34, factor VIII-related antigen.

Natural history
More common in R lobe
- probably because there is more R than L liver
Accelerated growth at puberty, during pregnancy or with OCP
- led to oestrogen-driven growth theory
--> however, this has not been proven
- but some do have estrogen receptors.

Complications
Majority are asymptomatic and discovered incidentally.
- all complications quite uncommon.
Don't rupture, don't cause cancer, don't remove.
Pain as above
Haemorrhage
- spontaneous rupture is extremely rare, like 0.2%
Inflammation; ?due to clotting and necrosis
- systemic symptoms possible
Mass effect, e.g. compression or displacement of GI organs or biliary tract.
- esp L lobe lesions.
- segments IVb, V and/or VI --> can compress duodenum or biliary tract.
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MANIFESTATIONS

Symptoms

Mainly asymptomatic and discovered incidentally on imaging
Pain / discomfort
- can be referred to shoulder
Mass effects on GI tract / bile duct
Nausea and vomiting

Signs
Observe
Dark red-purple, soft hypervascular lesions.
Usually well demarcated
Surrounded by a 'pseudocapsule' that facilitates enucleation.
Palpate
Mass
Hepatomegaly
Percuss
Hepatomegaly
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INVESTIGATIONS

Imaging
Characteristic features make diagnosis straightforward.
USS
Well circumscribed, homogenous hyper-echoic signal.
- can contain dense areas with fibrosis or calcification.
MRI
Best but also least accessible / most expensive.
Bright on T2
- typical peripheral enhancement after contrast.
Sensitivity and specificity ~90%.
CT
Often missed on non-contrast CT due to isodense appearance.
Typical peripheral enhancement after contrast.
- then enhancement progressively centrally; fills in

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MANAGEMENT

Rule out more serious conditions as cause of pain and appearance.
- then reassurance
- do not usually require surgery

Indications
Severe symptoms
Giant size with mass effect
Growth behavior.
Diagnostic uncertainty.
Spontaneous / traumatic rupture
- interventional embolization may be required first
Kasabach Merritt Syndrome (platelet sequestration).

Surgery
Safe by Cochrane r/v; mortality 0.4%
No guidelines.
But most prefer enucleation along with pseudocapsule in an avascular plane.
--> fewer complications, maximal preservation of liver tissue
But deep lesions may be better managed through anatomic resection.
Pringle manoeuvre may be very helpful
- limits blood loss.
- 30m usually well tolerated without ischaemia-reperfusion; 5m releases between re-clamping
- gentle pressure on tumour during reperfusion to prevent refilling and re-expansion.
Donor livers with larger haemangiomas are typically enucleated then implanted.
Treat raw surface with argon beam and can choose to fill with omentum.

Conservative
Nonoperative treatment.

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REFERENCES
Cameron 10th