Division of Surgical Oncology, Department of Surgery, Cedars Sinai Medical Center, Los Angeles, CA, USA.
Section of Hepatobiliary Tumors, Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center & Research Institute, 12902 Magnolia Drive, Tampa, FL, 33612, USA.
Curr Treat Options Oncol. 2020 Aug 29;21(11):88. doi: 10.1007/s11864-020-00784-2.
Small bowel neuroendocrine tumors (SB NETs) are increasing in frequency and becoming more common in surgical practice. It is often difficult to make the diagnosis of a SB NET at an early stage, as the primary tumor tends to be small and patients are asymptomatic until there is regional or distant metastasis, when they develop abdominal pain, partial obstruction, or bleeding and/or develop carcinoid syndrome. Despite this advanced presentation at the time of diagnosis, patients with metastatic SB NETs, as compared to other gastrointestinal malignancies, have favorable survival, which can be improved by appropriate surgical interventions. With the lack of randomized studies, there is reasonable controversy surrounding the optimal management of patients with SB NETs. As such, treatment of these patients is driven primarily by physician experience and available data based predominantly on retrospective studies. Based on this, current recommendations advocate for patients with SB NETs (localized or metastatic) to be managed at experienced centers by a multidisciplinary team. Eligible patients should undergo surgical resection of primary and regional disease as outlined in this article. Additionally, patients with metastatic disease should be evaluated on a case by case basis to evaluate surgical options that may mitigate bowel symptoms (i.e., pain, intestinal angina, obstruction) and carcinoid symptoms (flushing, diarrhea, hemodynamic instability) and prolong survival. Unlike other gastrointestinal malignancies, aggressive surgical management of these patients, even in the context of unresectable metastatic disease, can improve patients' symptoms and long-term survival. The principles outlined in this article are geared to guide appropriate management of SB NET patients with improvement in quality of life and overall survival outcomes.
小肠神经内分泌肿瘤 (SB NET) 的发病率正在增加，在外科实践中也越来越常见。由于原发肿瘤往往较小，且患者在出现局部或远处转移之前无症状，直到出现腹痛、部分梗阻、出血和/或类癌综合征时才会出现症状，因此早期诊断 SB NET 往往具有挑战性。尽管在诊断时已经处于晚期，但与其他胃肠道恶性肿瘤相比，转移性 SB NET 患者的生存情况较好，适当的手术干预可以改善其生存情况。由于缺乏随机研究，对于 SB NET 患者的最佳治疗方法存在合理的争议。因此，这些患者的治疗主要取决于医生的经验和主要基于回顾性研究的可用数据。基于此，目前的建议主张由多学科团队在有经验的中心对 SB NET 患者（局部或转移性）进行管理。符合条件的患者应按照本文所述接受原发和区域疾病的手术切除。此外，应根据具体情况评估转移性疾病患者的手术选择，这些选择可能减轻肠道症状（即疼痛、肠绞痛、梗阻）和类癌症状（潮红、腹泻、血流动力学不稳定）并延长生存时间。与其他胃肠道恶性肿瘤不同，即使在转移性疾病不可切除的情况下，对这些患者进行积极的手术治疗也可以改善患者的症状和长期生存。本文中概述的原则旨在指导 SB NET 患者的适当管理，以提高生活质量和总体生存结果。
