Kos-Kudła Beata, Ćwikła Jarosław, Ruchała Marek, Hubalewska-Dydejczyk Alicja, Jarzab Barbara, Krajewska Jolanta, Kamiński Grzegorz
Division of Endocrinology, Department of Pathophysiology and Endocrinology, Silesian Medical University, Katowice, Poland.
Department of Medical Science, University of Varmia & Masuria, Olsztyn, Poland.
Contemp Oncol (Pozn). 2017;21(2):115-122. doi: 10.5114/wo.2017.68619. Epub 2017 Jun 30.
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a large and very diverse group of neoplasms. Clinical presentation of NETs depends on the site of the primary tumor and whether the tumor is functioning (i.e., secreting peptides or neuroamines that produce symptoms). The diagnosis of GEP-NET is further complicated by symptomatic differences that occur depending on the type of secreted peptide or neuroamine. Due to their heterogeneity and unique characteristics, early diagnosis of GEP-NETs is difficult, which increases the likelihood of metastatic disease and reduces the scope of therapeutic possibilities. Thus, a multidisciplinary approach for the treatment of GEP-NETs is necessary. This review is the result of presentations that were delivered during an expert meeting on the treatment of GEP-NETs supported by Ipsen. We summarize the current knowledge on the epidemiology, incidence, diagnosis, and treatment of GEP-NETs. We examined the role of the somatostatin analog (SSA) lanreotide and the impact of the data from the recently published, randomized, double-blind, placebo-controlled CLARINET study (Controlled study of Lanreotide Antiproliferative Response In Neuroendocrine Tumors) on disease management. We also review the recent treatment options and recommendations for GEP-NETs.
胃肠胰神经内分泌肿瘤(GEP-NETs)是一大类且极为多样的肿瘤。神经内分泌肿瘤的临床表现取决于原发肿瘤的部位以及肿瘤是否具有功能(即分泌产生症状的肽类或神经胺类物质)。GEP-NET的诊断因所分泌的肽类或神经胺类物质类型不同而出现的症状差异进一步复杂化。由于其异质性和独特特征,GEP-NETs的早期诊断困难,这增加了发生转移性疾病的可能性并缩小了治疗选择范围。因此,采用多学科方法治疗GEP-NETs很有必要。本综述是在益普生支持的GEP-NETs治疗专家会议期间所做报告的成果。我们总结了目前关于GEP-NETs的流行病学、发病率、诊断和治疗的知识。我们研究了生长抑素类似物(SSA)兰瑞肽的作用以及最近发表的随机、双盲、安慰剂对照的CLARINET研究(神经内分泌肿瘤中兰瑞肽抗增殖反应对照研究)数据对疾病管理的影响。我们还综述了GEP-NETs的近期治疗选择和建议。