Sindhi Rakesh, Rohan Vinayak, Bukowinski Andrew, Tadros Sameh, de Ville de Goyet Jean, Rapkin Louis, Ranganathan Sarangarajan
Hillman Center for Pediatric Transplantation, UPMC-Children's Hospital of Pittsburgh, Pittsburgh, PA 15224, USA.
Medical University of South Carolina, Charleston, SC 29403, USA.
Cancers (Basel). 2020 Mar 19;12(3):720. doi: 10.3390/cancers12030720.
Unresectable hepatocellular carcinoma (HCC) was first removed successfully with total hepatectomy and liver transplantation (LT) in a child over five decades ago. Since then, children with unresectable liver cancer have benefitted greatly from LT and a confluence of several equally important endeavors. Regional and trans-continental collaborations have accelerated the development and standardization of chemotherapy regimens, which provide disease control to enable LT, and also serve as a test of unresectability. In the process, tumor histology, imaging protocols, and tumor staging have also matured to better assess response and LT candidacy. Significant trends include a steady increase in the incidence of and use of LT for hepatoblastoma, and a significant improvement in survival after LT for HCC with each decade. Although LT is curative for most unresectable primary liver sarcomas, such as embryonal sarcoma, the malignant rhabdoid tumor appears relapse-prone despite chemotherapy and LT. Pediatric liver tumors remain rare, and diagnostic uncertainty in some settings can potentially delay treatment or lead to the selection of less effective chemotherapy. We review the current knowledge relevant to diagnosis, LT candidacy, and post-transplant outcomes for these tumors, emphasizing recent observations made from large registries or larger series.
五十多年前，一名儿童通过全肝切除术和肝移植（LT）首次成功切除了不可切除的肝细胞癌（HCC）。从那时起，患有不可切除肝癌的儿童从LT以及几项同样重要的努力的融合中受益匪浅。区域和跨大陆合作加速了化疗方案的开发和标准化，这些方案可控制疾病以实现LT，同时也可作为不可切除性的测试。在此过程中，肿瘤组织学、成像方案和肿瘤分期也已成熟，以更好地评估反应和LT候选资格。显著趋势包括肝母细胞瘤的发病率和LT使用量稳步上升，以及HCC患者LT后的生存率每十年都有显著提高。尽管LT对大多数不可切除的原发性肝肉瘤（如胚胎性肉瘤）具有治愈性，但恶性横纹肌样瘤尽管接受了化疗和LT，仍容易复发。儿童肝肿瘤仍然罕见，在某些情况下诊断的不确定性可能会延迟治疗或导致选择效果较差的化疗。我们回顾了与这些肿瘤的诊断、LT候选资格和移植后结果相关的当前知识，重点强调了来自大型登记处或更大系列的最新观察结果。
