de Freitas Paganoti G, Tannuri A C A, Dantas Marques A C, Torres R R, Mendes Gibelli N E, Tannuri U
Pediatric Surgery Division, Pediatric Liver Transplantation Unit and Laboratory of Research in Pediatric Surgery (LIM 30), University of Sao Paulo Medical School, Sao Paulo, Brazil.
Pediatric Surgery Division, Pediatric Liver Transplantation Unit and Laboratory of Research in Pediatric Surgery (LIM 30), University of Sao Paulo Medical School, Sao Paulo, Brazil.
Transplant Proc. 2019 Jun;51(5):1605-1610. doi: 10.1016/j.transproceed.2019.03.004.
Surgery is a key factor in the treatment of hepatoblastoma, but choosing between an aggressive resection and liver transplant may be an extremely difficult task. The aim of this study was to describe the outcomes of patients with advanced hepatoblastoma: pretreatment extent of disease (PRETEXT)/post-treatment extent of disease (POST-TEXT) III and IV undergoing aggressive resections or living donor liver transplant in cases involving the entire liver. Based on this experience, a new protocol for the treatment of these patients was proposed.
A retrospective study included patients with advanced hepatoblastoma (POST-TEXT III and IV) who were referred for a liver transplant from 2010 to 2017.
A total of 24 children were included: 13 (54.2%) were male, with a median age at diagnosis of 42 months (range, 15-120 months), and a history of prematurity was identified in 20.8% of the patients. Ten cases (41.7%) were staged as PRETEXT/POST-TEXT III, and 12 cases (50.0%) were staged as PRETEXT/POST-TEXT IV. Two patients were referred after posthepatectomy recurrence. Five patients underwent a liver transplant, with recurrence and death in 2 patients (40.0%) within a mean period of 6 months. In the extensive hepatectomy group, there was recurrence in 6 patients (31.6%), with disease-free outcomes and overall survival in 63.2% and 94.7% of patients, respectively.
In cases of advanced hepatoblastoma, an extensive surgical approach is a valuable option. The fact that the team was fully prepared to proceed with living donor liver transplant allowed the surgeon to be more aggressive and to switch to transplantation when resection was not possible.
手术是肝母细胞瘤治疗的关键因素，但在积极切除和肝移植之间做出选择可能是一项极其困难的任务。本研究的目的是描述晚期肝母细胞瘤患者的治疗结果：疾病治疗前范围（PRETEXT）/疾病治疗后范围（POST - TEXT）为III期和IV期且在全肝受累情况下接受积极切除或活体肝移植的患者。基于这一经验，提出了一种针对这些患者的新治疗方案。
一项回顾性研究纳入了2010年至2017年因肝移植而转诊的晚期肝母细胞瘤（POST - TEXT III期和IV期）患者。
共纳入24名儿童：13名（54.2%）为男性，诊断时的中位年龄为42个月（范围15 - 120个月），20.8%的患者有早产史。10例（41.7%）分期为PRETEXT/POST - TEXT III期，12例（50.0%）分期为PRETEXT/POST - TEXT IV期。2例患者在肝切除术后复发后转诊。5例患者接受了肝移植，2例患者（40.0%）在平均6个月内复发并死亡。在广泛肝切除组中，6例患者（31.6%）复发，患者的无病生存率和总生存率分别为63.2%和94.7%。
在晚期肝母细胞瘤病例中，广泛的手术方法是一种有价值的选择。团队充分准备好进行活体肝移植这一事实使外科医生在无法进行切除时能够更积极地采取行动并转而进行移植。
