Endocrinology Unit, Regina Elena National Cancer Institute, Via Elio Chianesi, 53, Rome 00144, Italy.
J Exp Clin Cancer Res. 2010 Mar 2;29(1):19. doi: 10.1186/1756-9966-29-19.
Gastroenteropancreatic neuroendocrine tumours (GEP NETs) are rare tumours that present many clinical features.They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects.In 2000 the WHO developed a new classification which gives a better description of the characteristics and biological behaviour of the tumour.Surgical resection is the treatment of first choice for a patient with a GEP NET. In metastatic disease multiple therapeutic approaches are possible. In these cases the goal is to improve quality of life and to extent survival.GEP NETs express somatostatin receptors (SSTRs), which are bound by somatostatin (SST) or its synthetic analogues, although the subtypes and number of SSTRs expressed is very variable.Somatostatin analogues are used frequently to control hormone-related symptoms while their anti-neoplastic activity, even if it has not been widely studied and the regarding data are discordant, seems to result prevalently in tumour stabilisation.A few patients who fail to respond or cease to respond to standard SST analogues treatment seem to have a response to higher doses of these drugs.The use of higher doses of somatostatin analogues or the development of new subtype selective agonists and chimaeric somatostatin analogues, or pan-somatostatin will probably improve the clinical management of these patients.This review provides an update on the use of somatostatin analogues in the management of GEP NETs and discusses novel clinical strategies based on SSTR 2 gene transfer therapy.
胃肠胰神经内分泌肿瘤(GEP NETs)是一种罕见的肿瘤,具有多种临床表现。它们分泌肽类和神经胺,导致独特的临床综合征,包括类癌综合征。然而,许多肿瘤在出现肿块效应之前没有明显的临床表现,直到晚期才出现。2000 年,世界卫生组织(WHO)制定了一种新的分类,更好地描述了肿瘤的特征和生物学行为。手术切除是 GEP NET 患者的首选治疗方法。对于转移性疾病,有多种治疗方法可供选择。在这些情况下,治疗的目标是提高生活质量并延长生存时间。GEP NET 表达生长抑素受体(SSTRs),这些受体被生长抑素(SST)或其合成类似物结合,尽管表达的 SSTR 亚型和数量非常多样。生长抑素类似物常用于控制与激素相关的症状,尽管其抗肿瘤活性(尽管尚未广泛研究,相关数据存在分歧)似乎主要导致肿瘤稳定。少数对标准 SST 类似物治疗无反应或停止反应的患者似乎对这些药物的高剂量有反应。使用更高剂量的生长抑素类似物或开发新的亚型选择性激动剂和嵌合生长抑素类似物,或泛生长抑素可能会改善这些患者的临床管理。本文综述了生长抑素类似物在 GEP NET 治疗中的应用,并讨论了基于 SSTR2 基因转移治疗的新临床策略。