Iyer Renuka, Phan Alexandria T, Boudreaux J Philip
Liver and Pancreas Tumor Center, Roswell Park Cancer Institute, Buffalo, New York.
Gastrointestinal and Hepatobiliary Malignancy Programs, University of New Mexico Cancer Center, Albuquerque, New Mexico.
Clin Adv Hematol Oncol. 2017 Apr;15 Suppl 4(4):1-24.
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare malignancies that originate in the gastrointestinal system. GEP-NETs are typically indolent, but tumors known as "functional" secrete hormones that can lead to a complex of symptoms, including flushing, diarrhea, bronchospasm, and valvular heart disease. Management of patients with GEP-NETs requires a multidisciplinary approach, as treatment modalities include surgery, radiology, and pharmacotherapy. The available pharmacologic agents have increased in recently, and now include cytotoxic chemotherapies, somatostatin analogues, multitargeted tyrosine kinase inhibitors, mammalian target of rapamycin inhibitors, and radioisotopic radiotherapies. The optimal sequencing of treatments is unknown. Advances in the management of GEP-NETs have been based on the results of recently completed clinical trials that have shown improvement in disease outcome and symptom management. The amount of positive data that has emerged from these studies is unprecedented in the GEP-NETs field. At the 2017 American Society of Clinical Oncology Gastrointestinal Cancers Symposium, several abstracts provided subanalyses of previous trials and new data for emerging treatments. Management will likely evolve as these therapies are incorporated into clinical care.
胃肠胰神经内分泌肿瘤(GEP-NETs)是起源于胃肠系统的罕见恶性肿瘤。GEP-NETs通常生长缓慢,但被称为“功能性”的肿瘤会分泌激素,可导致一系列症状,包括潮红、腹泻、支气管痉挛和瓣膜性心脏病。GEP-NETs患者的管理需要多学科方法,因为治疗方式包括手术、放射学和药物治疗。最近可用的药物有所增加,现在包括细胞毒性化疗、生长抑素类似物、多靶点酪氨酸激酶抑制剂、雷帕霉素哺乳动物靶点抑制剂和放射性同位素放疗。治疗的最佳顺序尚不清楚。GEP-NETs管理方面的进展基于最近完成的临床试验结果,这些试验显示疾病结局和症状管理有所改善。这些研究中出现的阳性数据数量在GEP-NETs领域是前所未有的。在2017年美国临床肿瘤学会胃肠癌研讨会上,几篇摘要提供了对先前试验的亚分析以及新出现治疗方法的新数据。随着这些疗法被纳入临床护理,管理方式可能会不断演变。