Holzer K
Sektionsleitung Endokrine Chirurgie, Klinik für Viszeral‑, Thorax- und Gefäßchirurgie, Universitätsklinikum Marburg, Baldingerstraße, 35043, Marburg, Deutschland.
Chirurg. 2018 Jun;89(6):422-427. doi: 10.1007/s00104-018-0632-3.
Small (<2 cm) sporadic neuroendocrine pancreatic neoplasms (pNENs) are a heterogeneous group of neoplasms, which can be separated into functionally active or non-functional tumors. Functionally active pNENs release various hormones, such as insulin, gastrin, glucagon and vasoactive intestinal hormones and therefore lead to severe symptoms independent of their size. The main symptoms include hypoglycemia, coma (insulinoma), diarrhea (gastrinoma, VIPoma), intestinal ulcers (gastrinoma) and hyperglycemia (glucagonoma). Asymptomatic inactive pNENs do not cause a hormonal syndrome even though in immunohistochemistry they are positive for various hormones and endocrine markers (e.g. chromogranin and synaptophysin). Non-active small pNENs are increasingly being found because of the widespread use of high-resolution imaging and are named incidental pNENs. The current therapy algorithms of small functionally active and non-functional pNENs are very different and include besides non-operative management and surveillance (wait and see strategy) of non-active pNENs (G1, low G2), all minimally invasive, open and robotic techniques of pancreas resection. Until today almost all recommendations of surgical and nonsurgical therapy algorithms have been established based on retrospective data.
小（<2厘米）的散发性胰腺神经内分泌肿瘤（pNENs）是一组异质性肿瘤，可分为功能性活跃或无功能性肿瘤。功能性活跃的pNENs会释放各种激素，如胰岛素、胃泌素、胰高血糖素和血管活性肠激素，因此无论其大小都会导致严重症状。主要症状包括低血糖、昏迷（胰岛素瘤）、腹泻（胃泌素瘤、血管活性肠肽瘤）、肠道溃疡（胃泌素瘤）和高血糖（胰高血糖素瘤）。无症状的无功能性pNENs即使在免疫组织化学中对各种激素和内分泌标志物（如嗜铬粒蛋白和突触素）呈阳性，也不会引起激素综合征。由于高分辨率成像的广泛应用，越来越多地发现无功能性小pNENs，它们被称为偶发性pNENs。目前，小的功能性活跃和无功能性pNENs的治疗算法非常不同，除了对无功能性pNENs（G1，低G2）进行非手术管理和监测（观察等待策略）外，还包括胰腺切除的所有微创、开放和机器人技术。直到今天，几乎所有手术和非手术治疗算法的建议都是基于回顾性数据建立的。
