Outcome of Children Treated for Infantile Hepatic Hemangioendothelioma

Abstract

Purpose: Infantile hepatic hemangioendothelioma (IHHE) is the most common hepatic vascular tumor in children. We report on the treatment outcome of our large single-center experience of patients with IHHE over a 9-year period. Materials and Methods: A retrospective analysis of all IHHE patients treated at the Children Cancer Hospital Egypt from April 2008 through April 2017. Results: In total, 28 patients (18 females, 10 males) were diagnosed with IHHE with a median age at diagnosis of 3 months. The lesions were multifocal (n=12), focal (n=10), and diffuse (n=6). Six (21.4%) patients initially had low T3 and T4. Eleven patients did not receive any treatment, whereas 1 patient underwent resectional surgery. Sixteen patients received drug treatment, 9 of whom responded well to first-line propranolol/prednisolone, whereas 7 patients needed salvage treatment. Twenty-five patients are alive, whereas 3 patients have died. Conclusions: Overall, patients with IHHE do well, a significant percentage of whom do not require drug therapy, particularly for those with small focal lesions. In patients with multifocal/diffuse disease, there is a high incidence of low T3 and T4 and while some of these patients did well without additional therapy, those with rapidly progressive lesions during treatment may do poorly.

Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report

“…Our present case showed a high level of AFP, but the diagnosis of HCC was not taken into account, and the multiple hepatic tumors were considered as infantile hemangioendothelioma. Infantile hepatic hemangioendothelioma is the most common hepatic vascular tumor in children [ 12 ]. In a single-center experience of 28 cases, the median age at diagnosis was 3 months.…”

“…Sixteen patients received drug treatment, nine of whom responded well to first-line propranolol/prednisolone, whereas seven patients needed salvage treatment. Twenty-five patients are alive, whereas three patients have died [ 12 ]. In 15 adult cases of hepatic epithelioid hemangioendothelioma who underwent hepatic resection or LT at our institution, no patient showed elevated AFP levels at all [ 13 , 14 ].…”

Hepatic Epithelioid Hemangioendothelioma Presenting Synchronously with Hepatocellular Carcinoma

“…Thomas et al [14] reported similar 5-year OS rates for EHE patients who underwent surgery, chemotherapy, embolization, or observation. Additionally, observation without treatment shows favorable outcomes in pediatric EHE cases [15]. However, definitive biomarkers of EHE aggressiveness have not been established.…”

Primary bilateral angiosarcoma of the breast treated with neoadjuvant chemotherapy combined with propranolol

“…Basing on our own experience in adult patients treatment gained in oncology clinic and on pediatric literature regarding the role of the beta-adrenergic receptor blockade in low-aggressive vascular tumors (hemangioendothelioma epitheloides), 5,6 it was decided to administer the patient with 10 mg of propranolol three times a day. On cross section in the inner quadrants, a lesion 5 × 2.7 × 1.5 cm in diameter was visible, removed with a margin of adjacent tissues: the narrowest margin on the fascia side 2.5 cm wide, from the top 4.5 cm, from the bottom 3.5 cm.…”