Report of Hepatoblastoma in One of Monochorionic Diamniotic Twins With Extremely Low Birth Weight

Abstract

We present the case of a patient with hepatoblastoma, born at 24 weeks and 6 days of gestation and weighting 568 g, and one of the monochorionic diamniotic spontaneous twins. She was the donor of twin-to-twin transfusion syndrome. She showed a hepatic enlargement during the 7th month checkup and was referred to us. Abdominal CT scan showed a large mass in the right hepatic lobe and she was diagnosed as having PRETEXT II hepatoblastoma. Following neoadjuvant chemotherapy, she underwent right hepatectomy. The twins were diagnosed as having Beckwith-Wiedemann Syndrome (BWS) by molecular genetic testing. Although the risk of tumor development based on BWS was similar between them, hepatoblastoma was found only in the affected twin. The affected twin was born with a lower birth weight, received oxygen therapy longer, and used diuretics longer than the nonaffected twin. This case suggests that the prenatal environment and postnatal intensive care differences are associated with the development of hepatoblastoma.