Department of Surgery, Division of Surgical Oncology, Yale School of Medicine, New Haven, CT, USA.
VA Connecticut Healthcare System, West Haven, CT, USA.
Curr Treat Options Oncol. 2024 Sep;25(9):1137-1152. doi: 10.1007/s11864-024-01207-2. Epub 2024 Jul 31.
Gastric neuroendocrine neoplasms (G-NENs) are a heterogeneous group of tumors that broadly fall into two groups. The first group, driven by oversecretion of gastrin, are generally multifocal, small, and behave indolently with a low (but non-zero) risk of progression and metastatic spread. They are conventionally categorized into type 1, with endogenous gastric-based overproduction of gastrin, and type 2 G-NEN, with overproduction of gastrin from an extra-gastric gastrin-secreting tumor. The second group, termed type 3 G-NEN, occur spontaneously and are potentially more aggressive, having a clinical course analogous to other neuroendocrine tumors of the gastrointestinal tract. Type 1 G-NEN can be managed with endoscopic surveillance and resection of visible lesions with great success, reserving surgery for the rare high-risk lesion, whereas surgical resection of the causative gastrin-secreting tumor in type 2 G-NEN is usually curative. Type 3 G-NEN is usually managed with formal surgical resection but there is growing evidence that limited surgery or even endoscopic resection in appropriately selected patients with low risk is both safe and effective. A novel subtype of G-NEN, associated with long-term proton pump inhibitor usage, is increasing in incidence. The pathophysiology seems to parallel type 1 G-NEN. In the setting of metastatic disease, which can occur in any subtype but is most common by far in type 3 G-NEN, the lack of trial data unique to G-NEN results in extrapolation of strategies and agents for treatment of non-gastric neuroendocrine disease. The rapid pace of development in this area is likely to benefit the metastatic G-NEN patient as well. As treatment is predicate on type of G-NEN, establishing the etiology of the lesion is crucial but growing knowledge of G-NEN pathophysiology and close collaboration between pathologists, gastroenterologists, radiologists, surgeons, and oncologists have enabled a growing trend towards de-escalation and less-invasive treatment paradigms.
胃神经内分泌肿瘤（G-NENs）是一组异质性肿瘤，广泛分为两类。第一类肿瘤受胃泌素过度分泌驱动，通常为多灶性、小肿瘤，且生长缓慢，进展和转移扩散的风险较低（但并非为零）。它们通常被归类为 1 型，即胃内源性胃泌素过度产生，和 2 型 G-NEN，即胃泌素由胃外分泌胃泌素的肿瘤过度产生。第二类，称为 3 型 G-NEN，自发性发生，且潜在侵袭性更强，其临床病程与胃肠道其他神经内分泌肿瘤类似。1 型 G-NEN 可以通过内镜监测和切除可见病变来成功管理，仅将手术保留给极少数高危病变；而 2 型 G-NEN 中分泌胃泌素的肿瘤的手术切除通常是治愈性的。3 型 G-NEN 通常通过正式手术切除来管理，但越来越多的证据表明，对于低风险的适当选择患者，有限的手术甚至内镜切除既安全又有效。一种与长期质子泵抑制剂使用相关的新型 G-NEN 亚型的发病率正在增加。其病理生理学似乎与 1 型 G-NEN 相似。在转移性疾病的情况下，任何亚型都可能发生，但到目前为止，3 型 G-NEN 最为常见，由于缺乏针对 G-NEN 的独特试验数据，导致对非胃神经内分泌疾病的治疗策略和药物的推断。该领域的快速发展也可能使转移性 G-NEN 患者受益。由于治疗取决于 G-NEN 的类型，因此确定病变的病因至关重要，但对 G-NEN 病理生理学的认识不断提高以及病理学家、胃肠病学家、放射科医生、外科医生和肿瘤学家之间的密切合作，使逐渐减少侵袭性和非侵入性治疗方案的趋势得以发展。
