Etiology: there are no clear factors in the development of this tumor
Prevalence: very rare.
Sonographic findings: large heterogeneous masses (up to 15cm), generally unique. It has predilection for the posterior section of the right lobe. It may contain calcifications. The spectral Doppler demonstrates both arterial and venous signals. Drainage veins can be seen exiting the tumor. The hepatic artery and celiac axis were dilated with a small aorta under the celiac axis. The fetus may present with either oligo or polyhydramnios.
Differential diagnosis: mesenchymal hamartoma, adenoma, nodular hyperplasia, metastasis of the neuroblastoma.
Associated anomalies: Cardiac failure from A-V shunting and non-immune-hydrops. Hepatomegalia, cutaneous hemangioma
Prognosis: Disseminated intravascular coagulation can result from platelet sequestration (syndrome of Kasabach-Merrit). Spontaneous fetal death can occur due to the hydrops. Spontaneous involution between 6 and 8 months has been described too.
Management: Treatment by radiotherapy or with corticoids may reduce the tumor by vasoconstriction. If the lesion is unresponsive, embolization or ligature of the collateral veins and the hepatic artery can be attempted. Currently, prenatal treatment with corticoids is attempted.