Management of undifferentiated sarcoma of the liver including living donor liver transplantation as a backup procedure

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• 作者：Hideaki Okajima ; Yuki Ohya ; Kwang-Jong Lee ; Hidekazu Yamamoto ; Katsuhiro Asonuma ; Yuko Nagaoki ; Kazunori Ohama ; Masahiko Korogi ; Tadashi Anan ; Motohiro Hashiyama ; Fumio Endo ; Kenichi Iyama ; Yukihiro Inomata
• 关键词：Undifferentiated sarcoma of the liver ; Living donor liver transplantation
• 刊名：Journal of Pediatric Surgery
• 出版年：2009
• 出版时间：February 2009
• 年：2009
• 卷：44
• 期：2
• 页码：e33-e38
• 全文大小：663 K

文摘

We present the cases of 3 children with huge undifferentiated sarcoma of the liver who were treated with surgical excision including liver transplantation as an option and adjuvant chemotherapy. All 3 patients were males aged 10, 13, and 15 years old. The size of the tumor was 10, 15, and 20 cm in diameter, respectively. The youngest patient is disease free and doing well 43 months after resection. The 13-year-old patient presented with tumor rupture and underwent operation. The primary tumor and the ruptured tissue fragments were removed and he was given postoperative chemotherapy. The patient is disease free and doing well 52 months after surgery. The oldest patient had an unresectable tumor in the hilar region. Preoperative chemotherapy was given but later discontinued owing to severe side effects. He underwent living donor liver transplantation followed by postoperative chemotherapy. The patient had recurrent tumor 24 months after transplantation that was excised at reoperation. He is doing well and is disease free 18 months after the second procedure. Complete removal of the tumor including total hepatectomy and transplantation when indicated and suitable pre- and/or postoperative chemotherapy is an effective treatment for children with undifferentiated sarcoma of the liver.