Pediatric hepatic angiosarcoma treated with chemotherapy and partial hepatectomy: A case report.

Abstract

e21001 Background: Hepatic angiosarcoma is a high-grade malignant neoplasm, extremely rare in children and associated with a poor prognosis. It is reported in English language less than fifty cases in children between 2 months and 15- year-old at presentation. Due to the rarity of this tumor type, little is known about effective systemic therapy. Many diferent schedules have been reported, comprising surgery only, neoadjuvant chemotherapy, surgery wih adjuvant radiotherapy and hepatic transplantation. The median survival rate stays between 10 to 48 months, independently of the treatment chosen, with inconsistent results. Methods: retrospective medical records review. Objective: We present our experience wih a female 3-year-old child presenting wih a huge unressectable liver angiosarcoma at diagnosis, treated with neoadjuvant intensive chemotherapy based on ifosfamide, carboplatine and etoposide and right hepatectomy. The child remained in complete remission for 5 years, after that presenting with local relapse treated again with the same intensive chemotherapy, resulting in a very good response. Conclusion: the children is alive after a relapsed hepatic angiosarcoma treated with neoadjuvant chemotherapy and partial hepatectomy. In this case, neoadjuvant chemotherapy based in carboplatine, ifofamide and etoposide was an effective strategy to treat pediatric hepatic angiosarcoma.

Pediatric Primary Hepatic Tumors: Diagnostic Considerations

“…There is a reported female predominance within the cases, and the median age is reported to be 40 months [ 62 , 65 , 66 ]. We were able to find 72 published cases of pediatric hepatic angiosarcoma since 1944 ( Supplemental Table S1 ) [ 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 , 85 , 86 , 87 ].…”