A Case of Neonatal Giant Hepatic Hemangioendothelioma With Kasabach-Merritt Syndrome

Abstract

We experienced a case of neonatal giant hepatic hemangioendothelioma. The abdominal mass was detected by fetal echogram at 35 weeks gestation in this patient. The patient was delivered by Caesarean section at 36 weeks and 3 days gestation. The patient was intubated and received mechanical ventilation just after birth because she had a respiratory disorder due to the giant abdominal mass. Blood, examinations revealed anemia, thrombocytopenia and prolonged coagulation time, which indicate disseminated intravascular coagulation (DIG). Ultrasonography, CT scan and an MRI revealed a large hepatic mass with dilatation of left hepatic vein. Under a diagnosis of hepatic hemangioma with Kasabach-Merritt syndrome, steroid and radiation therapies were performed, resulting in the improvement of DIG. But there was little change in the tumor size, so we performed a tumor resection at 60 days, after identifying the tumor as hepatic origin by angiography. The histology of the tumor was hemangioendothelioma. The patient was discharged 3 weeks after the operation. It is likely that surgical resection after steroid administration and radiation is an effective and safe treatment for neonatal giant hemangioendothelioma when complicated with a condition such as DIG.