Clinical experience with infantile hepatic hemangioendothelioma.

Abstract

Infantile hepatic hemangioendothelioma (IHHE) is a rare disorder with only a few series reported in the medical literature. We reviewed our treatment experience with IHHE over 17 years. A retrospective analysis of patients with IHHE between 1991 and 2008 was performed. Sixteen patients (median age 30 days) with IHHE were identified. A palpable abdominal mass was the most common presentation. All except two cases could be diagnosed radiologically. Thirteen patients had a unilobar single tumor and three patients had bilobar disease. Nine patients with symptoms and a resectable tumor underwent complete resection. Three patients with symptoms and unresectable tumor underwent medical treatment with steroids and interferon. Four asymptomatic patients were closely observed. Overall, 14 patients were cured and 1 patient died of postoperative bleeding. One patient is still on medication, and the tumor has greatly decreased in size. Two patients with bilobar disease showed elevated levels of serum alpha-fetoprotein at presentation. Histopathology confirmed type 1 IHHE in all of the 10 specimens. The presence of clinical symptoms is a key element determining the treatment options. In symptomatic patients, primary surgical resection should be considered whenever feasible.