Hepatic hemangioendothelioma

Guillermina M. Ochua, MD, Ricardo De Loredo , MD, Roque A. Carpio , MD, Javier B. Gardey, MD, Silvina Fernàndez, MD

Hospital Privado del Sur, Servicio de Ecograf├Ča, Las Heras 164, Bah├Ča Blanca, C├▓digo postal: 8000 Provincia de Buenos Aires, Argentina

Synonyms: Hepatic arteriovenous malformation.

Definition: Benign neoplasia of vascular origin.

Case Report: This is a 36-year-old G1P1A1 patient who had her first ultrasound at 36 weeks.  The examination demonstrated a singleton fetus with normal cardiac activity and fetal movement. The fetal biometry corresponded to the fiftieth percentile of 36 weeks (Hadlock). The placenta was anterior. A mixed mass was seen with well-defined edges in left fetal hemiabdomen. The mass displaced the umbilical cord. The fetal stomach and left suprarenal could not be seen. The amniotic fluid was normal.

Prenatal images of the hemangioendotelioma. At the superior level of the abdomen, the desplacement of the umbilical artery by a mixed mass. The right superrenal is normal while the left superrenal and the fetal stomach are not seen.

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Postnatal examination of the liver immediately after birth.

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Postnatal tomography without intravenous contrast. The hipodense, heterogeneous lesion can be seen in the left lobe of the liver which takes the periferal contraste to the center, suggesting the disagnosis of a hemangioendotelioma.

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Postnatal examination one and a half months after birth. The corticords treatment began, showing evidence of a shrinking tumor.

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Postnatal examination four months after birth. The tumor is reduced with corticord treatment.

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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.

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