Clinical case series of pediatric hepatic angiosarcoma

Abstract

Hepatic angiosarcoma is a rare, aggressive, malignant neoplasm with fewer than 50 cases reported in children. Prognosis is poor, with a minority surviving beyond 2 years after diagnosis. We report eight cases of pediatric hepatic angiosarcoma, diagnosed at a mean age of 3 years. Seven were initially diagnosed with an infantile hepatic hemangioendothelioma (IHHE) or hemangioma and the eighth with a "vascular tumor." Two patients, who received liver transplant, survived. We suggest hepatic hemangiomas can rarely transform into angiosarcomas and a subset of IHHEs (Type II) are actually a low-grade form of angiosarcoma rather than a benign lesion.

Success of chemotherapy and a liver transplant in a pediatric patient with hepatic angiosarcoma: A case report

“…Results are mixed, but overall outcomes and prognosis remain very poor. Platinum‐based chemotherapy regimens have often been tried, as well as targeted therapy with sirolimus and, more recently, with vascular endothelial growth factor inhibitors such as bevacizumab . Sizable tumor response to bevacizumab, gemcitabine, and docetaxel was reported in a case of metastatic HAS but as most commonly seen, the child ultimately died after 16 cycles .…”

“…After complete resection and over a year of alternating courses of varying cytotoxic agents, the patient remained disease‐free 44 months post‐diagnosis . Sclerotherapy, directed embolization, and radiation have also been attempted, with little success . While many different agents have been tried with varying benefit as briefly referenced above, the majority never achieve remission or long‐term survival.…”

“…Hepatic angiosarcoma is a rare diagnosis in pediatric patients, more often seen in adults, and can be difficult to distinguish from other vascular anomalies such as type II IHE. Malignant liver tumors account for approximately 1% of childhood malignancies with hepatic angiosarcoma representing only 1%‐2% of pediatric liver tumors . There are fewer than 50 pediatric cases reported in the literature worldwide .…”

“…Malignant liver tumors account for approximately 1% of childhood malignancies with hepatic angiosarcoma representing only 1%‐2% of pediatric liver tumors . There are fewer than 50 pediatric cases reported in the literature worldwide . The overall prognosis is very poor, regardless of treatment or stage, with mean survival time ranging from just 10 months to 2 years .…”

“…There are fewer than 50 pediatric cases reported in the literature worldwide . The overall prognosis is very poor, regardless of treatment or stage, with mean survival time ranging from just 10 months to 2 years . There are only five cases of disease‐free survival reported in the literature …”