Maurizi Angela, Partelli Stefano, Falconi Massimo
Pancreatic Surgery Unit, AOUI Ospedali Riuniti - Università Politecnica delle Marche, Ancona, Italy.
Front Horm Res. 2015;44:139-48. doi: 10.1159/000382143. Epub 2015 Aug 14.
Pancreatic neuroendocrine tumors (pNETs) are rare and clinically demanding diseases. They are clinically defined as functioning or nonfunctioning depending on the presence of a syndrome related to inappropriate hormone secretion. Functioning pNETs are often small in size and localization may be difficult also due to their possible extraduodenal-pancreatic origin in the jejunum, stomach, mesentery, spleen and ovaries. The improvement and the widespread use of cross-sectional imaging techniques has significantly increased the detection of small, nonfunctioning pNETs. The European Neuroendocrine Tumor Society (ENETS) guidelines now suggest a 'wait and see' policy for these small, incidentally discovered tumors, which are smaller than 2 cm. On the other hand, surgery still always represents the treatment of choice for pNETs >2 cm and/or for symptoms. A large proportion of patients with pNETs have locally advanced disease at diagnosis. Radical surgery of pNETs includes both typical and atypical pancreatic resections. At diagnosis, between 25 and 93% of patients with pNETs have synchronous neuroendocrine tumor liver metastases. Radical resection of the primary tumor, associated with complete, eventually multistep, resection of the liver metastases, can be considered in selected cases. In recent years minimally invasive approaches, either laparoscopic or robotic, have played an increasing role in the treatment of pNETs.
胰腺神经内分泌肿瘤(pNETs)是罕见且临床治疗颇具挑战的疾病。根据是否存在与激素分泌不当相关的综合征,临床上将其定义为功能性或非功能性。功能性pNETs通常体积较小,由于其可能起源于空肠、胃、肠系膜、脾脏和卵巢等十二指肠外胰腺部位,定位也可能困难。横断面成像技术的改进和广泛应用显著提高了小的非功能性pNETs的检出率。欧洲神经内分泌肿瘤学会(ENETS)指南现建议对这些偶然发现的小于2 cm的小肿瘤采取“观察等待”策略。另一方面,对于直径>2 cm和/或有症状的pNETs,手术仍然始终是首选治疗方法。很大一部分pNETs患者在诊断时已出现局部晚期疾病。pNETs的根治性手术包括典型和非典型胰腺切除术。在诊断时,25%至93%的pNETs患者存在同步性神经内分泌肿瘤肝转移。在某些选定病例中,可考虑对原发肿瘤进行根治性切除,并对肝转移灶进行完整的、最终可能是多步骤的切除。近年来,微创方法,无论是腹腔镜还是机器人手术,在pNETs治疗中发挥着越来越重要的作用。