A 2.6-kg boy was born at 33 weeks' gestation after an uncomplicated pregnancy. Fetal sonography at 18 weeks' gestation was reportedly normal. At delivery, the infant was noted to have a left upper quadrant mass. The patient was otherwise normal and did not have respiratory distress. Sonography ( Figure 1) and computerized tomography ( Figure 2) of the abdomen were obtained. A large mass in the left lobe of liver with peripheral calcification and a cystic or necrotic center was demonstrated by these studies. Thrombocytopenia was present with a platelet count of 40,000. Hematocrit and hemoglobin were normal. The -fetoprotein was remarkably elevated at 46,023 ng/ml ( <400 ng/ml normal in newborns). Initial chest radiograph was normal. The patient was taken to the operating room for resection of the hepatic mass after adequate platelet levels were obtained by transfusion.
DENOUEMENT AND DISCUSSIONThe pathologic specimen of the left hepatic lobe demonstrated an infantile hemangioendothelioma. The peripheral portion of the mass contained scattered dystrophic calcifications. The large central portion demonstrated only abundant hemorrhagic necrosis. Platelet levels slowly returned to normal postoperatively.Infantile hepatic hemangioendothelioma (IHHE) is the most common vascular lesion of the liver in newborns. 1 The classic clinical findings consist of the triad of cutaneous hemangiomas, congestive heart failure, and hepatomegaly.1 Arteriovenous shunting often occurs within IHHE and is the cause of the congestive heart failure, anemia, and thrombocytopenia. Infants with congestive heart failure and consumptive coagulopathy with thrombocytopenia have a poor prognosis.1 Approximately 85% of IHHE are diagnosed before 6 months of age. Although the tumors increase in size initially after birth, they typically involute during the first year of life. In most cases, because of the clinical setting and arteriovenous shunting, the diagnosis of IHHE is straightforward.At sonography, IHHE may be hypo-, iso-, or hyperechoic to adjacent parenchyma. Small lesions tend to be homogeneous, whereas larger lesions are heterogeneous and may have a necrotic center. The classic computerized tomography finding is centripetal enhancement after intravenous contrast.The presence of extensive cystic necrosis and elevated levels of -fetoprotein requires consideration of additional lesions, in particular, cystic mesenchymal hamartoma and a necrotic hepatoblastoma.2 Cystic mesenchymal hamartoma is usually multiloculated and found in the right lobe of the liver in children less than 2 years of age.3 Although the lesions may be solid, 93% are at least partially cystic.4 -Fetoprotein levels are elevated in many cases with the range 3200 to 6000 ng/ml.5 Calcifications are uncommon in most series.3 However, lesions with increased peripheral vascularity around cysts are often confused with IHHE.