Department of Surgery, Campus Charité Mitte | Campus Virchow-Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany.
Leiter Arbeitsbereich endokrine Chirurgie, Chirurgische Klinik, Campus Charité Mitte | Campus Virchow-Klinikum, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.
Rev Endocr Metab Disord. 2018 Jun;19(2):169-178. doi: 10.1007/s11154-018-9469-9.
The incidence of neuroendocrine tumors (NET) increases with age. Lately, the diagnosis of NET was mainly caused by early detection of small NET (<1 cm) in the rectum and stomach, which are depicted by chance during routine and prophylactic endoscopy. Also in patients with large and metastatic pancreatic and intestinal tumors thorough pathologic investigation with use of different immunohistologic markers discovers more neuroendocrine tumors with low differentiation grade (G2-G3) and more neuroendocrine carcinomas (NEC), nowadays, than in former times. While gastric and rectal NET are discovered as small (<1 cm in diameter) and mainly highly differentiated tumors, demonstrating lymph node metastases in less than 10% of the patients, the majority of pancreatic and small bowel NET have already metastasized at the time of diagnosis. This is of clinical importance, since tumor stage and differentiation grade not only influence prognosis but also surgical procedure and may define whether a combination of surgery with systemic biologic therapy, chemotherapy or local cytoreductive procedures may be used. The indication for surgery and the preferred surgical procedure will have to consider personal risk factors of each patient (i.e. general health, additional illnesses, etc.) and tumor specific factors (i.e. tumor stage, grade of differentiation, functional activity, mass and variety of loco regional as well as distant metastases etc.). Together they define, whether radical curative or only palliative surgery can be applied. Altogether surgery is the only cure for locally advanced NET and helps to increase quality of life and overall survival in many patients with metastatic neuroendocrine tumors. The question of cure versus palliative therapy sometimes only can be answered with time, however. Many different aspects and various questions concerning the indication and extent of surgery and the best therapeutic procedure are still unanswered. Therefore, a close multidisciplinary cooperation of colleagues involved in biochemical and localization diagnostics and those active in various treatment areas is warranted to search for the optimal strategy in each individual patient. How far genetic screening impacts survival remains to be seen. Since surgeons do have a central role in the treatment of NET patients, they have to understand the need for integration into such an interdisciplinary team.
神经内分泌肿瘤（NET）的发病率随着年龄的增长而增加。最近，NET 的诊断主要是由于直肠和胃中早期发现的小 NET（<1cm），这些 NET 是在常规和预防性内窥镜检查中偶然发现的。此外，在患有大的和转移性胰腺和肠道肿瘤的患者中，使用不同的免疫组织化学标志物进行彻底的病理检查，发现更多低分化程度（G2-G3）和更多神经内分泌癌（NEC）的神经内分泌肿瘤，这比以前要多。虽然胃和直肠 NET 被发现为小（<1cm 直径）且主要是高度分化的肿瘤，在不到 10%的患者中显示淋巴结转移，但大多数胰腺和小肠 NET 在诊断时已经转移。这具有临床重要性，因为肿瘤分期和分化程度不仅影响预后，而且还影响手术程序，并可能确定是否可以将手术与系统生物治疗、化疗或局部细胞减灭术相结合。手术的适应证和首选手术程序将不得不考虑每位患者的个人风险因素（即一般健康状况、其他疾病等）和肿瘤特异性因素（即肿瘤分期、分化程度、功能活动、肿块大小和局部区域以及远处转移等）。它们共同决定是否可以进行根治性或姑息性手术。总的来说，手术是局部晚期 NET 的唯一治愈方法，有助于提高许多转移性神经内分泌肿瘤患者的生活质量和总体生存率。然而，有时只有随着时间的推移才能回答治愈还是姑息治疗的问题。关于手术的适应证和范围以及最佳治疗程序仍有许多未解决的问题。因此，涉及生化和定位诊断的同事以及在各种治疗领域活跃的同事之间需要密切的多学科合作，以寻找每个患者的最佳策略。基因筛查对生存的影响还有待观察。由于外科医生在 NET 患者的治疗中起着核心作用，因此他们必须理解需要融入这种跨学科团队。
