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理解分化良好的胰腺神经内分泌肿瘤的管理和治疗:临床医生应对复杂疾病的指南。

Understanding the Management and Treatment of Well-Differentiated Pancreatic Neuroendocrine Tumors: A Clinician's Guide to a Complex Illness.

机构信息

Department of Medical Oncology, City of Hope National Medical Center, Duarte, CA.

Department of Hematology and Medical Oncology, Harbor-UCLA Medical Center, Torrance, CA.

出版信息

JCO Oncol Pract. 2020 Nov;16(11):720-728. doi: 10.1200/JCOOP.20.00010. Epub 2020 Oct 21.

DOI:10.1200/JCOOP.20.00010
PMID:33085933
Abstract

Pancreatic neuroendocrine tumors (PanNETs) are rare neoplasms that arise in the neuroendocrine cells of the pancreas. Although their clinical presentations differ depending on cell type, most are indolent, whereas others cause noteworthy hormone-related symptoms. The increasing incidence of PanNETs, attributed to improved diagnostic modalities, demonstrates advances in current standard of care. However, given the heterogeneity of these tumors, treatment decisions can become complex and an individualized approach is often required. Surgical intervention has remained the mainstay for localized tumors, whereas systemic therapies remain viable options for patients with unresectable or metastatic disease. Liver-directed therapies such as radiofrequency ablation and hepatic arterial embolization have also become available adjunct therapies for patients with liver-predominant metastases. Despite the increase in the armamentarium of treatment options for patients with PanNETs, data regarding the ideal sequence of treatment, especially systemic treatments, are currently lacking. Ongoing clinical trials are aimed at addressing this knowledge gap in addition to developing the next generation of novel therapeutics.

摘要

胰腺神经内分泌肿瘤(PanNETs)是一种罕见的肿瘤,起源于胰腺的神经内分泌细胞。虽然它们的临床表现因细胞类型而异,但大多数是惰性的,而其他则会引起明显的激素相关症状。PanNETs 的发病率不断上升,这归因于诊断方法的改进,表明当前的治疗标准有所进步。然而,鉴于这些肿瘤的异质性,治疗决策可能变得复杂,通常需要个体化的方法。手术干预仍然是局部肿瘤的主要治疗方法,而对于不可切除或转移性疾病的患者,全身治疗仍然是可行的选择。对于肝转移为主的患者,肝内定向治疗如射频消融和肝动脉栓塞术也已成为辅助治疗方法。尽管患者的 PanNETs 治疗选择方案有所增加,但关于治疗的理想顺序的数据,尤其是全身治疗方面的数据,目前仍然缺乏。正在进行的临床试验旨在解决这一知识空白,并开发下一代新型治疗药物。

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