Avila L F, Encinas J L, Leal N, Guinea A, García Miguel P, Jara P, Murcia J, Gamez M, Guinea A, López Santamaría M, Tovar J A
Departamento de Cirugía Pediátrica, Hospital Infantil Universitario La Paz, Madrid.
Cir Pediatr. 2007 Oct;20(4):189-93.
To analyse our results on liver transplantation (LTX) in primitive malignant unresectable liver tumours in children and discussing its controversial indications in order to our experience.
METHODS/PATIENTS: We report 12 patients with ages ranging from 6 months to 14 years old. They had hepatoblastoma (11) and fibrolamellar hepatocelullar carcinoma (1) without cirrhosis. LTX was considered as primary treatment in 10 patients (PRETEXT IV or any grade if extension to retrohepatic cava vein, 3 hepatic veins or porta vein were assessed) and as rescue therapy after recurrence (1) or persistence of unresectable macroscopic rests (2). One of the patients who underwent a LTX as primary therapy had lung metastases previously resolved with chemotherapy. We used entire liver (5), left lateral segment from cadaveric donor (3), live related donor (3, 2 segments II-III and 1 right liver) and left lateral segment from split (1). All children received chemotherapy prior and post-transplantation following SIOPEL protocol. OUTCOMES ANALYSED: Procedure tolerance, survival, recurrence rate, disease-free period and risk factors for adverse evolution.
All patients overcame the LTX and no early loss of the graft was assessed. 2 patients died because of tumoral relapse, 1 after primary LTX and 1 after rescue LTX (survival rate of both groups 90% vs 50%). Graft and patients 1-year, 3-year, 5-year and 14-year survival were 91%, 91%, 82% and 82% respectively. The boy who presented lung metastases developed new ones one year after LTX that were removed and he actually is free of disease. The disease-free period has a probability for 1, 3 and 5 years of 91%, 75% and 75%. Tumoral tissue persistence is the only risk factor for an adverse evolution in our series.
LTX is possible therapeutic approach for unresectable malignant liver tumours. It provides better results as a primary treatment than as a rescue one, being these outcomes comparable to those from resectable tumours. A right staging and referring patients to an expertise centre contribute to optimize results. LTX for patients presenting with lung metastases could be a controversial option. Live-related donor transplantation is an excellent alternative to avoid disease progression during cadaveric waiting list.
分析我们对儿童原发性不可切除恶性肝肿瘤进行肝移植（LTX）的结果，并结合我们的经验探讨其存在争议的适应证。
方法/患者：我们报告了12例年龄在6个月至14岁之间的患者。他们患有肝母细胞瘤（11例）和纤维板层肝细胞癌（1例），均无肝硬化。10例患者（PRETEXT IV期或如果评估肿瘤侵犯至肝后腔静脉、3支肝静脉或门静脉则为任何分期）将LTX作为主要治疗方法，1例在复发后、2例在不可切除的大体肿瘤残留持续存在后将LTX作为挽救治疗。1例接受LTX作为主要治疗的患者先前有肺转移，经化疗后已缓解。我们使用了全肝（5例）、尸体供体的左外叶（3例）、活体亲属供肝（3例，2例为II - III段，1例为右肝）和劈离式供肝的左外叶（1例）。所有儿童在移植前后均按照SIOPEL方案接受化疗。分析的结果：手术耐受性、生存率、复发率、无病生存期以及不良进展的危险因素。
所有患者均成功完成LTX，未评估到早期移植物丢失。2例患者因肿瘤复发死亡，1例在初次LTX后，1例在挽救性LTX后（两组生存率分别为90%对50%）。移植物和患者的1年、3年、5年和14年生存率分别为91%、91%、82%和82%。出现过肺转移的男孩在LTX后1年出现新的转移灶，已切除，目前无疾病。无病生存期1年、3年和5年的概率分别为91%、75%和75%。肿瘤组织残留是我们系列中不良进展的唯一危险因素。
LTX是治疗不可切除恶性肝肿瘤的一种可行的治疗方法。作为主要治疗方法比作为挽救治疗效果更好，这些结果与可切除肿瘤的结果相当。正确的分期并将患者转诊至专业中心有助于优化结果。对有肺转移的患者进行LTX可能是一个有争议的选择。活体亲属供肝移植是避免在尸体供肝等待名单期间疾病进展的一个极佳选择。
