Faraj Walid, Dar Faisal, Marangoni Gabriele, Bartlett Adam, Melendez Hector Vilca, Hadzic Dino, Dhawan Anil, Mieli-Vergani Georgina, Rela Mohamed, Heaton Nigel
Institute of Liver Studies, King's College Hospital, School of Medicine, King's College London, London, UK.
Liver Transpl. 2008 Nov;14(11):1614-9. doi: 10.1002/lt.21586.
From October 1993 to February 2007, 25 liver transplantations were performed for hepatoblastoma. Of these 25, 18 children received cadaveric grafts, and 7 received left lateral segments from living donors. Fifteen patients were at level IV in the pretreatment extent of disease staging system for hepatoblastoma (PRETEXT IV; 11 received cadaveric grafts and 4 underwent living related liver transplantation [LRLT]) and 10 were level III (PRETEXT III; 7 received cadaveric grafts and 3 underwent LRLT). Preoperative chemotherapy was given according to the risk stratification system for children with hepatoblastoma protocols of the International Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL): SIOPEL I in the first 3 patients, SIOPEL II in 6, SIOPEL III in 10, and SIOPEL IV in 3 patients. Patient and graft survival after cadaveric transplantation was 91%, 77.6%, and 77.6%, at 1, 5, and 10 years, respectively, with no retransplantations. Patient and graft survival for children undergoing LRLT was 100%, 83.3%, and 83.3%, at 1, 5, and 10 years, respectively. All surviving children but 1 remain disease-free, with a median follow up of 6.8 years (range, 0.9-14.9). There were 5 deaths at a median of 13 months post-transplantation, secondary to tumor recurrence (4) and respiratory failure (1). Liver transplantation is an established treatment for unresectable hepatoblastoma confined to the liver following chemotherapy. LRLT is a therapeutic option given that the outcome is similar to that of resection and cadaveric transplantation.
1993年10月至2007年2月，共进行了25例肝母细胞瘤肝移植手术。这25例中，18例儿童接受了尸体供肝移植，7例接受了活体供者的左外叶肝段移植。15例患者在肝母细胞瘤术前疾病分期系统（PRETEXT）中处于IV期（PRETEXT IV；11例接受尸体供肝移植，4例接受活体亲属肝移植[LRLT]），10例处于III期（PRETEXT III；7例接受尸体供肝移植，3例接受LRLT）。术前化疗根据国际小儿肿瘤学会（SIOPEL）国际儿童肝肿瘤策略组制定的肝母细胞瘤患儿风险分层系统进行：前3例患者采用SIOPEL I方案，6例采用SIOPEL II方案，10例采用SIOPEL III方案，3例采用SIOPEL IV方案。尸体供肝移植术后1年、5年和10年的患者及移植物生存率分别为91%、77.6%和77.6%，均未进行再次移植。接受LRLT的儿童患者及移植物生存率在1年、5年和10年分别为100%、83.3%和83.3%。除1例患者外，所有存活儿童均无疾病复发，中位随访时间为6.8年（范围0.9 - 14.9年）。移植术后中位13个月时有5例死亡，原因分别为肿瘤复发（4例）和呼吸衰竭（1例）。肝移植是化疗后局限于肝脏的不可切除肝母细胞瘤的既定治疗方法。鉴于LRLT的治疗效果与肝切除及尸体供肝移植相似，它是一种治疗选择。
