Gastrointestinal Surgery and Liver Transplantation, Fondazione IRCCS Istituto Nazionale Tumori (National Cancer Institute), Via Venezian 1, 20133, Milan, Italy.
University of Milan, Milan, Italy.
Rev Endocr Metab Disord. 2017 Dec;18(4):473-483. doi: 10.1007/s11154-017-9439-7.
Liver metastases occur in nearly half of NET patients (MNETs) and heavily affect prognosis, with 5-yr. OS around 19-38%. Although it is difficult to show outcome differences for available treatments, due to the long course of disease, surgery for MNETs remains the most effective option in terms of survival and symptom control. Since MNETs frequently present as an oligo-metastatic, liver-limited disease, unresectable in 80% of cases, liver transplantation (LT) has emerged as a potential curative treatment. Nevertheless, experience with LT for MNETs is limited and burdened by highly heterogeneous outcomes and significant recurrence rate, mostly explained by the variability of selection criteria. Several prognostic factors have been identified: extended surgery on primary tumor associated to LT, elderly patients, pancreatic primary (pNET), extensive liver involvement, poorly differentiated tumors, high Ki67 levels and short wait time to LT. A proper patients' selection based on these data (Milan NET criteria) allows a significant survival advantage over non-transplant strategies, with excellent outcomes in recent series (69-97.2% 5-yr. OS) as opposed to patients undergoing non-surgical treatments (34-50.9%). Evidence indicates LT as the best option for selected patients with MNETs. The use of organs for MNETs is therefore justified.
近一半的神经内分泌肿瘤(NET)患者会发生肝转移,这严重影响了预后,5 年总生存率(OS)约为 19%-38%。尽管由于疾病的病程较长,很难显示出现有治疗方法的疗效差异,但对于 MNET 患者,手术仍然是生存和症状控制方面最有效的选择。由于 MNET 通常表现为寡转移、肝局限性疾病,80%的病例无法切除,因此肝移植(LT)已成为一种潜在的根治性治疗方法。然而,LT 治疗 MNET 的经验有限,且结局高度异质,复发率高,这主要与选择标准的差异有关。已经确定了一些预后因素:与 LT 相关的原发肿瘤的广泛手术、老年患者、胰腺原发(pNET)、广泛的肝受累、分化差的肿瘤、高 Ki67 水平和 LT 等待时间短。根据这些数据(米兰 NET 标准)进行适当的患者选择,与非移植策略相比,可显著提高生存率,在最近的系列研究中,5 年 OS 率为 69%-97.2%,而接受非手术治疗的患者为 34%-50.9%。有证据表明 LT 是 MNET 患者的最佳选择。因此,使用器官治疗 MNET 是合理的。