神经内分泌肿瘤(NETs)代表一组异质性肿瘤,它们的原发肿瘤部位多种多样,功能状态(即激素分泌或非功能性)和侵袭程度(从分化良好,1级神经内分泌肿瘤到低分化3级神经内分泌癌)。最常见的部位是肺,小肠,胰腺和阑尾。临床表现是可变的,从横断面成像检测到的附带病变,小肠梗阻,类癌综合征或其他综合征表现(如胰岛素瘤引起的低血糖),直至类癌心脏病。诊断依赖于生化标志物,计算机断层扫描(CT),磁共振成像(MRI)和基于生长抑素受体的功能成像。治疗包括可以进行治愈性切除的手术,通过疾病稳定是关键的方法,涉及生长抑素类似物,肽受体放射性核素治疗(PRRT),依维莫司,舒尼替尼,肝定向治疗,有时化疗。尽管可能发生局部和全身并发症,它们与合理的5年和10年生存率相关,分别。
Neuroendocrine tumours (NETs) represent a heterogenous group of tumours, with diversity in their primary tumour sites, functional status (ie hormone secreting or non-functional) and degrees of aggressiveness (ranging from well-differentiated, grade 1 neuroendocrine tumours to poorly differentiated grade 3, neuroendocrine carcinomas). The most common sites are the lung, small bowel, pancreas and appendix. Clinical presentation is variable, ranging from incidental lesions detected on cross-sectional imaging, small bowel obstruction, carcinoid syndrome or other syndromic presentations (eg hypoglycaemia resulting from insulinoma) through to florid carcinoid heart disease. Diagnosis relies on biochemical markers, computed tomography (CT), magnetic resonance imaging (MRI) and somatostatin-receptor based functional imaging. Treatment comprises surgery where curative resection is possible through to approaches where disease stabilisation is the key, involving somatostatin analogues, peptide receptor radionuclide therapy (PRRT), everolimus, sunitinib, liver-directed therapies and sometimes chemotherapy. Although local and systemic complications can occur, they are associated with reasonable 5- and 10-year survival rates, respectively.