Shimata Keita, Sugawara Yasuhiko, Hibi Taizo
Department of Transplantation and Pediatric Surgery, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan.
Gland Surg. 2018 Feb;7(1):42-46. doi: 10.21037/gs.2017.12.11.
Patients with pancreatic neuroendocrine tumors (pNETs) very often present with a metastatic disease at the first diagnosis. Liver transplantation (LT) for unresectable pNET with liver metastases (pNETLM) has been described to prolong survival in highly selected patients, although outcomes were worse than those of patients who underwent LT for gastrointestinal NETLM (GI-NETLM). In this review, several proposed criteria are described with their rationale and controversies. Most of the data used to establish these criteria do not reflect the recent improvements of non-surgical treatments that has changed the landscape of treatment for pNETs, including the development of peptide receptor radionuclide therapy and molecular-targeted agents (sunitinib and everolimus). Properly designed studies are necessary to define the role of down-staging and bridging therapy prior to LT incorporating systemic chemotherapy using these molecular-targeted agents. Also, given the indolent nature of low or intermediate grade pNETs, the best endpoint to compare the efficacy of each treatment option for patients with pNETLM has yet to be determined. Lastly, the definition of "unresectable" remains ambiguous. The indication of the conventional technique of two-staged liver resection with portal vein embolization or the new technique of associating liver partition and portal vein ligation for staged hepatectomy to expand the resectability of wide-spread metastatic liver tumors has been controversial. In an era of transplant oncology, LT should be the last resort for patients who are considered unresectable and otherwise untreatable after an exhaustive multidisciplinary team discussion by all experts in the field. In conclusion, although its long-term outcomes have been promising, the role of LT for unresectable pNETLM as a curative or palliative treatment remains unclear. A well-designed randomized control study is required to elucidate the clinical impact of LT for pNETLM.
胰腺神经内分泌肿瘤(pNETs)患者在首次诊断时常常已出现转移性疾病。对于不可切除的伴有肝转移的pNET(pNETLM)患者,肝移植(LT)已被描述为可延长经过高度筛选患者的生存期,尽管其结果比接受肝移植治疗胃肠道神经内分泌肿瘤肝转移(GI-NETLM)的患者更差。在本综述中,描述了几种提出的标准及其原理和争议。用于确立这些标准的大多数数据并未反映出非手术治疗的近期进展,这些进展改变了pNETs的治疗格局,包括肽受体放射性核素治疗和分子靶向药物(舒尼替尼和依维莫司)的发展。需要进行设计合理的研究,以确定在LT之前使用这些分子靶向药物进行全身化疗的降期和桥接治疗的作用。此外,鉴于低级别或中级别的pNETs具有惰性,尚未确定比较pNETLM患者每种治疗方案疗效的最佳终点。最后,“不可切除”的定义仍不明确。采用门静脉栓塞的两阶段肝切除传统技术或联合肝脏分隔和门静脉结扎的分期肝切除新技术以扩大广泛转移性肝肿瘤的可切除性一直存在争议。在移植肿瘤学时代,对于那些在该领域所有专家进行详尽的多学科团队讨论后被认为不可切除且无法以其他方式治疗的患者,LT应作为最后手段。总之,尽管其长期结果令人期待,但LT作为不可切除的pNETLM的治愈性或姑息性治疗的作用仍不明确。需要进行精心设计的随机对照研究以阐明LT对pNETLM的临床影响。