Department of Pediatrics, The Warren Alpert Medical School of Brown University, Providence, RI 02903, USA.
J Pediatr Surg. 2009 Dec;44(12):e1-3. doi: 10.1016/j.jpedsurg.2009.09.008.
Undifferentiated embryonal sarcoma of the liver is a rare hepatic malignancy of childhood with a historically poor prognosis. Recent improvements in outcomes have been reported in small numbers of cases with the use of combination therapy involving aggressive surgical resection and chemotherapy. Complete surgical resection is frequently difficult to achieve when the location of the tumor is along the margins of the major hepatic vessels (portal vein, hepatic vein, and hepatic artery). Here we report a case of undifferentiated embryonal sarcoma of the liver that recurred along surgical hepatic vein margins in a 9-year-old boy who subsequently underwent orthotopic liver transplantation from a cadaveric donor. The patient has been in continuous clinical remission for the last 5 years.
肝未分化胚胎肉瘤是一种罕见的儿童肝脏恶性肿瘤,历史上预后较差。最近,在使用包括积极手术切除和化疗在内的联合治疗的少数情况下,已有报道称其治疗结果有所改善。当肿瘤位于大肝血管(门静脉、肝静脉和肝动脉)边缘时,通常很难完全切除肿瘤。在这里,我们报告了一例肝未分化胚胎肉瘤病例,该病例在 9 岁男孩的肝静脉手术边缘处复发,随后接受了来自尸体供体的原位肝移植。患者在过去 5 年内一直持续临床缓解。