Reyes J D, Carr B, Dvorchik I, Kocoshis S, Jaffe R, Gerber D, Mazariegos G V, Bueno J, Selby R
University of Pittsburgh, Thomas E. Starzl Transplantation Institute, PA, USA.
J Pediatr. 2000 Jun;136(6):795-804.
To describe our experience with total hepatectomy and liver transplantation as treatment for primary hepatoblastoma (HBL) and hepatocellular carcinoma (HCC) in children.
A retrospective analysis of the perioperative course of 31 children with unresectable primary HBL (n = 12) and HCC (n = 19) who underwent transplantation between May 1989 and December 1998. Systemic (n = 18) and intraarterial (n = 7) neoadjuvant chemotherapy were administered; follow-up ranged from 1 to 185 months.
For HBL, 1-year, 3-year, and 5-year posttransplantation survival rates were 92%, 92%, and 83%, respectively. Intravenous invasion, positive hilar lymph nodes, and contiguous spread did not have a significant adverse effect on outcome; distant metastasis was responsible for 2 deaths. Intraarterial chemotherapy was effective in all patients treated. For HCC, the overall 1-year, 3-year, and 5-year disease-free survival rates were 79%, 68%, and 63%, respectively. Vascular invasion, distant metastases, lymph node involvement, tumor size, and gender were significant risk factors for recurrence. Intraarterial chemotherapy was effective in 1 of 3 patients. Six patients died of recurrent HCC, and 3 deaths were unrelated to recurrent tumor.
Liver transplantation for unresectable HBL and HCC can be curative. Risk factors for recurrence were significant only for HCC, with more advanced stages amenable to cure in the HBL group.
描述我们采用全肝切除及肝移植治疗儿童原发性肝母细胞瘤(HBL)和肝细胞癌(HCC)的经验。
对1989年5月至1998年12月间接受移植的31例无法切除的原发性HBL(n = 12)和HCC(n = 19)患儿的围手术期过程进行回顾性分析。给予全身化疗(n = 18)和动脉内化疗(n = 7);随访时间为1至185个月。
对于HBL,移植后1年、3年和5年生存率分别为92%、92%和83%。静脉侵犯、肝门淋巴结阳性和连续扩散对预后无显著不良影响;远处转移导致2例死亡。动脉内化疗对所有接受治疗的患者均有效。对于HCC,总体1年、3年和5年无病生存率分别为79%、68%和63%。血管侵犯、远处转移、淋巴结受累、肿瘤大小和性别是复发的显著危险因素。动脉内化疗在3例患者中有1例有效。6例患者死于复发性HCC,3例死亡与复发性肿瘤无关。
对于无法切除的HBL和HCC,肝移植可治愈。仅HCC存在复发的危险因素,HBL组中更晚期的病例也可治愈。