Pediatric Hepatic Angiosarcoma: Case Report and Review of the Literature

Abstract

Pediatric hepatic angiosarcoma (PHAS) is a rare tumor, which usually presents as a rapid enlargement of the liver. To date, surgery, chemotherapy, and radiotherapy have not improved the poor prognosis of PHAS with only three survivors reported. The histology of PHAS is distinct from adult angiosarcoma, because PHAS displays hypercellular whorls of sarcomatous cells, or "kaposiform" spindle cells, in addition to the general features of angiosarcoma. We report a case of PHAS that was treated with vascular ablation, chemotherapy, and liver transplantation. Lung metastases occurred 14 months posttransplant.

Malignant vascular tumors of liver in neonates

“…An association with environmental exposure to Thorotrast, arsenic, vinyl chloride, androgenic and anabolic steroids, and oral contraceptives is rarely reported in pediatric age [13]. It is more common in young girls and the mean age of presentation reported in literature is 40 months [3,9,14]. The most frequent presentation is a painless abdominal mass, which may be accompanied by anemia, jaundice, consumptive coagulopathy, and rarely spontaneous hemoperitoneum.…”

“…Malignant transformations of infantile hemangioendothelioma after successful medical treatment are documented in literature [9,11,12]. Type 2 infantile hemangioendotheliomas are now considered a form of hepatic angiosarcoma [1,3,4,9,11]. Patient 1 was probably an example of this rare presentation of angiosarcoma because there was no response to aggressive medical treatment and lung metastasis appeared in later part of clinical course.…”

Differential diagnosis and management of liver tumors in infants

“…If an hepatic hemangioma shows an unusual progression, malignancy should be suspected and a tumor biopsy is warranted. Hepatic angiosarcoma is a rare and highgrade malignant neoplasm that accounts for 2% of liver tumors in children [55][56][57] . Early metastatic disease to the lungs is commonly seen.…”

Liver Tumors in Children

“…These tumors occur in children 5-10 years of age and are mesenchymal in appearance. While the vast majority of vascular tumors of the liver in childhood are benign hemangioendotheliomas, angiosarcoma of the liver is a particularly aggressive malignant subtype with a poor prognosis [15,16]. Embryonal rhabdomyosarcomas arise from biliary ducts and usually arise in children Ͻ5 years of age [17].…”