Sadowski Samira M, Triponez Frederic
1 Endocrine Oncology Branch, National Institutes of Health, Bethesda, MD 20892, USA ; 2 Thoracic and Endocrine Surgery, University Hospitals of Geneva, 1211 Geneva, Switzerland.
Gland Surg. 2015 Feb;4(1):63-8. doi: 10.3978/j.issn.2227-684X.2014.12.01.
Pancreatic neuroendocrine tumors (PNETs) are frequent and can be non-functional (NF) in patients with multiple endocrine neoplasia type 1 (MEN1). Their identification is of clinical importance because malignant PNETs are reported to be the most common cause of death in patients with MEN1. Once the diagnosis of MEN1 is established in an individual based on clinical manifestations and/or genetic testing results, an active surveillance program is instituted for early detection and treatment of MEN1-associated disease. Ultrasonography, endoscopic ultrasonography (EUS), CT, MRI, selective arterial angiography and somatostatin receptor scintigraphy are all used for localization of tumors. Managing PNETs can be challenging and includes diagnosis, surveillance, adequate staging, and interdisciplinary, multimodal treatments to optimize patient outcome. Treatment includes surgical resection for loco-regional disease, as well as liver directed and targeted chemotherapies for advanced progressive disease. To date, the recommendation for surgical resection in NF-PNETs is based on tumor size, as a higher rate of metastases was found in patients with larger tumors. This review summarizes key concepts in managing PNETs in patients with MEN1.
胰腺神经内分泌肿瘤（PNETs）很常见，在1型多发性内分泌腺瘤病（MEN1）患者中可能为无功能型（NF）。它们的识别具有临床重要性，因为据报道恶性PNETs是MEN1患者最常见的死亡原因。一旦根据临床表现和/或基因检测结果在个体中确立了MEN1的诊断，就会启动一个积极的监测计划，以早期发现和治疗与MEN1相关的疾病。超声、内镜超声（EUS）、CT、MRI、选择性动脉血管造影和生长抑素受体闪烁显像均用于肿瘤定位。管理PNETs可能具有挑战性，包括诊断、监测、充分分期以及跨学科、多模式治疗，以优化患者预后。治疗包括针对局部区域疾病的手术切除，以及针对晚期进展性疾病的肝靶向和靶向化疗。迄今为止，NF-PNETs手术切除的建议基于肿瘤大小，因为在肿瘤较大的患者中发现转移率较高。本综述总结了MEN1患者管理PNETs的关键概念。
