La Salvia Anna, Espinosa-Olarte Paula, Riesco-Martinez Maria Del Carmen, Anton-Pascual Beatriz, Garcia-Carbonero Rocío
Oncology Department, Hospital Universitario 12 de Octubre, UCM, 28041 Madrid, Spain.
Cancers (Basel). 2021 Apr 3;13(7):1701. doi: 10.3390/cancers13071701.
Neuroendocrine tumors (NETs) are a heterogeneous family of neoplasms of increasing incidence and high prevalence due to their relatively indolent nature. Their wide anatomic distribution and their characteristic ability to secrete hormonally active substances pose unique challenges for clinical management. They are also characterized by the common expression of somatostatin receptors, a target that has been extremely useful for diagnosis and treatment (i.e., somatostatin analogues (SSAs) and peptide-receptor radionuclide therapy (PRRT)). Chemotherapy is of limited use for NETs of non-pancreatic origin, and the only approved targeted agents for advanced progressive NETs are sunitinib for those of pancreatic origin, and everolimus for lung, gastrointestinal and pancreatic primaries. Despite recent therapeutic achievements, thus, systemic treatment options remain limited. In this review we will discuss the state-of-the-art targeted therapies in the field of NETs, and also future perspectives of novel therapeutic drugs or strategies in clinical development, including recently presented results from randomized trials of yet unapproved antiangiogenic agents (i.e., pazopanib, surufatinib and axitinib), PRRT including both approved radiopharmaceuticals (177Lu-Oxodotreotide) and others in development (177Lu-Edotreotide, 177Lu-Satoreotide Tetraxetan), immunotherapy and other innovative targeted strategies (antibody-drug conjugates, bites,…) that shall soon improve the landscape of personalized treatment options in NET patients.
神经内分泌肿瘤（NETs）是一类异质性肿瘤，由于其相对惰性的性质，发病率和患病率不断上升。它们广泛的解剖分布以及分泌具有激素活性物质的独特能力给临床管理带来了独特挑战。它们还具有生长抑素受体共同表达的特征，这一靶点对诊断和治疗（即生长抑素类似物（SSAs）和肽受体放射性核素治疗（PRRT））极为有用。化疗对非胰腺来源的NETs作用有限，而对于晚期进展性NETs唯一获批的靶向药物，对于胰腺来源的是舒尼替尼，对于肺、胃肠道和胰腺原发肿瘤的是依维莫司。尽管最近取得了治疗成果，但全身治疗选择仍然有限。在本综述中，我们将讨论NETs领域的最新靶向治疗方法，以及临床开发中新型治疗药物或策略的未来前景，包括最近未获批的抗血管生成药物（即帕唑帕尼、索凡替尼和阿昔替尼）随机试验的结果、PRRT（包括已获批的放射性药物（177Lu-奥曲肽）和其他正在开发的药物（177Lu-依多曲肽、177Lu-司他瑞肽四醋酸盐））、免疫治疗以及其他创新靶向策略（抗体药物偶联物、双特异性抗体等），这些将很快改善NET患者个性化治疗选择的局面。
