The authors describe a 10-week-old girl with infantile hepatic hemangioendothelioma who initially presented with difficulty feeding, hepatomegaly, and multiple hemangiomas of the skin. Six weeks of steroid therapy and 2 weeks of chemotherapy failed to produce clinical improvement. The patient underwent split liver transplantation. A definitive diagnosis of hemangioendothelioma type II was made. Imaging studies cannot differentiate between hemangioendothelioma and angiosarcoma. Treatment modalities for this condition remain unclear. The patient continues to do well.
A 10-week-old white girl, born at 30 weeks' gestation, was referred to our hospital with difficulty feeding, poor weight gain, and abdominal distention. Examination was remarkable for significant hepatomegaly distending the abdomen, along with multiple cutaneous hemangiomas. There was no evidence of arteriovenous shunting or congestive heart failure. Ultrasonography (Fig. 1) and CT (Fig. 2) of the abdomen revealed multiple spherical hepatic lesions with circumferential enhancement. Significant laboratory findings included hemoglobin 8.9 g/dL, platelet count 630,000/mm, alpha-fetoprotein 14,226 (normal range 0.0-88 ng/mL), PT 14.1 seconds, and PTT 42.1 seconds (normal range 11-13 and 22-36 seconds, respectively). A clinical and radiologic diagnosis of infantile hepatic hemangioendothelioma (IHHE) was made. Treatment with prednisone (4 mg/kg per day) was commenced.
Sequential ultrasounds during 4 weeks of steroid therapy confirmed no change in the size of the liver. Cushingoid side effects prompted reduction of the steroid dose to 2.5 mg/kg per day. Six weeks after the initial presentation the patient was readmitted for decreasing oral intake, vomiting, and worsening abdominal distention. She was admitted to the pediatric intensive care unit for poor respiratory effort and was promptly intubated and placed on mechanical ventilation. Cyclophosphamide therapy was initiated at 10 mg/d for 5 days. The patient was started on Synthroid after thyroid stimulating hormone (TSH) levels were found to be 175 IU (normal range 0.4-4.7 mIU/L). The multicentric involvement of hepatic tissue in this case made local surgical resection impossible. Failure of steroid and chemotherapy and worsening clinical status led to the decision for orthotopic liver transplantation. The liver tumor continued to enlarge, impinging on lung expansion and necessitating emergency split-liver transplantation. Pathologic examination of the explanted liver revealed IHHE type II (Fig. 3).