Ramos-Gonzalez Gabriel, LaQuaglia Michael, O'Neill Allison F, Elisofon Scott, Zurakowski David, Kim Heung Bae, Vakili Khashayar
Department of Surgery, Boston Children's Hospital, Boston, MA, USA.
Dana-Farber Cancer Institute, Boston, MA, USA.
Pediatr Transplant. 2018 Jun 11:e13250. doi: 10.1111/petr.13250.
HB is the most common primary liver tumor in children. Complete tumor excision, either by partial resection or by total hepatectomy and liver transplantation, in combination with chemotherapy provides the best chance for cure. We performed a retrospective analysis of patients who underwent liver transplantation for HB and herein present our 14-year single-institution experience. Twenty-five patients underwent liver transplantation for HB at a median age of 26 months (IQR: 15-44). Graft survival was 96%, 87%, and 80% at 1, 3, and 5 years, respectively. There were four patient deaths, three of them due to disease recurrence within the first year post-transplant. Ten-year overall survival was 84%. Three recipients initially presented with pulmonary metastases and underwent resection of metastatic disease, of which two are alive at 3.9 years. Of three patients who underwent salvage transplants, two are alive at 1.5 years after transplant. Non-survivors were associated with lower median alpha fetoprotein value at presentation compared to survivors (21 707 vs 343 214; P = .04). In conclusion, the overall long-term outcome of primary liver transplantation for HB is excellent. Tumor recurrence was the highest contributor to mortality. Even patients with completely treated pulmonary metastases prior to transplant demonstrated a favorable survival.
肝母细胞瘤是儿童最常见的原发性肝脏肿瘤。通过部分切除或全肝切除及肝移植完整切除肿瘤,并结合化疗,提供了最佳的治愈机会。我们对因肝母细胞瘤接受肝移植的患者进行了回顾性分析,并在此介绍我们单机构14年的经验。25例患者因肝母细胞瘤接受肝移植,中位年龄为26个月(四分位间距:15 - 44个月)。1年、3年和5年的移植物存活率分别为96%、87%和80%。有4例患者死亡,其中3例死于移植后第一年内的疾病复发。10年总生存率为84%。3例最初表现为肺转移的受者接受了转移病灶切除术,其中2例在3.9年时仍存活。在3例接受挽救性移植的患者中,2例在移植后1.5年时仍存活。与存活者相比,非存活者在就诊时的中位甲胎蛋白值较低(21707 vs 343214;P = 0.04)。总之,肝母细胞瘤原发性肝移植的总体长期预后良好。肿瘤复发是导致死亡的最主要因素。即使是在移植前已完全治疗肺转移的患者也显示出良好的生存率。