Angelico Roberta, Grimaldi Chiara, Saffioti Maria Cristina, Castellano Aurora, Spada Marco
Division of Abdominal Transplantation and Hepatobiliopancreatic Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Division of Oncohematology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Transl Gastroenterol Hepatol. 2018 Sep 10;3:59. doi: 10.21037/tgh.2018.09.05. eCollection 2018.
Hepatocellular carcinoma (HCC) is a rare malignancy in children and at the time of diagnosis up to 80% of pediatric HCC are unresectable due to large and multiple lesions. The majority of pediatric HCC occurs on a background of normal liver, and consequently the absence of concomitant chronic liver disease generally allows tolerating pre- and post-operative chemotherapy. Based on the large experiences of adult HCC and pediatric hepatoblastoma, in the last years a multidisciplinary aggressive treatment composed of surgical resection and chemotherapy (based on cisplatin and doxorubicin) has been proposed, improving patient outcomes and recurrence rate in children with HCC. However, the overall survival rate in children with HCC is not satisfactory yet; while the 5-year survival rate may achieve up to 70-80% in non-metastatic resectable HCC, it remains <20% in children with unresectable HCC. The mainstay of the pediatric HCC therapeutic strategy is the radical tumor resection, weather by hepatic resection or liver transplantation, nevertheless the best surgical approaches as well as the optimal neoadjuvant and adjuvant treatment are still under debate. Different strategies have been explored to convert unresectable HCC into resectable tumors by extending criteria for surgical treatment and/or associating multi-modal treatments, such as systemic and local-regional therapy, but universal recommendation needs to be defined yet. The purpose of this review is to outline the role of different surgical approaches, including hepatic resection and liver transplantation, in pediatric HCC with or without underlying chronic liver disease.
肝细胞癌(HCC)在儿童中是一种罕见的恶性肿瘤,在诊断时,高达80%的儿童HCC由于病灶大且多发而无法切除。大多数儿童HCC发生在肝脏正常的背景下,因此,由于不存在慢性肝病,一般能够耐受术前和术后化疗。基于成人HCC和儿童肝母细胞瘤的大量经验,近年来提出了一种由手术切除和化疗(基于顺铂和阿霉素)组成的多学科积极治疗方法,改善了HCC患儿的治疗效果和复发率。然而,HCC患儿的总体生存率仍不尽人意;虽然非转移性可切除HCC的5年生存率可达70-80%,但不可切除HCC患儿的5年生存率仍低于20%。儿童HCC治疗策略的主要支柱是根治性肿瘤切除,无论是通过肝切除还是肝移植,然而,最佳的手术方法以及最佳的新辅助和辅助治疗仍存在争议。已经探索了不同的策略,通过扩大手术治疗标准和/或联合多模式治疗(如全身和局部区域治疗)将不可切除的HCC转化为可切除的肿瘤,但仍需要确定通用的推荐方案。本综述的目的是概述不同手术方法(包括肝切除和肝移植)在伴有或不伴有潜在慢性肝病的儿童HCC中的作用。