title: Angiosarcoma successfully treated with liver transplantation and sirolimus.
abstract: Malignant liver tumors represent approximately 1% of malignancies in children. HA is a high-grade tumor of endothelial cells that is even more rare in the pediatric population. HA has a limited response to chemotherapy, radiation and resection with universal tumor recurrence with LT and nearly 100% mortality by 18 months. This is the first reported successful case of hepatic angiosarcoma in a child who was treated by LT in combination with sirolimus. Sirolimus antagonizes the mTOR pathway, which regulates cell proliferation, differentiation, and migration, and is being studied as an anti-neoplastic agent for solid tumors.
authors.affiliation: Pediatrics Baylor College of Medicine, Houston, TX, USA.
authors.forename: Megan
authors.initials: M
authors.lastname: Xue
authors.forename: Prakash
authors.initials: P
authors.lastname: Masand
authors.forename: Patrick
authors.initials: P
authors.lastname: Thompson
authors.forename: Milton
authors.initials: M
authors.lastname: Finegold
authors.forename: Daniel H
authors.initials: DH
authors.lastname: Leung
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D005260:Female; D006394:Hemangiosarcoma / Q000188:drug therapy* / Q000601:surgery; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008113:Liver Neoplasms / Q000188:drug therapy* / Q000601:surgery; D016031:Liver Transplantation; D020123:Sirolimus / Q000627:therapeutic use*
chemical_list: D000903:Antibiotics, Antineoplastic; D007166:Immunosuppressive Agents; D020123:Sirolimus
keywords: child; liver transplantation; malignancy; pediatric liver transplantation; sirolimus