Department of Pathology, University Medical Center Utrecht, Utrecht University, Heidelberglaan 100, 3584 CX Utrecht, the Netherlands.
Department of Medicine, Boston University School of Medicine, 820 Harrison Avenue, FGH 2011, Boston, MA 02118, USA.
Surg Pathol Clin. 2022 Sep;15(3):541-554. doi: 10.1016/j.path.2022.05.007. Epub 2022 Aug 2.
Pancreatic neuroendocrine tumors (PanNETs) represent a clinically challenging disease because these tumors vary in clinical presentation, natural history, and prognosis. Novel prognostic biomarkers are needed to improve patient stratification and treatment options. Several putative prognostic and/or predictive biomarkers (eg, alternative lengthening of telomeres, alpha-thalassemia/mental retardation, X-linked (ATRX)/Death Domain Associated Protein (DAXX) loss) have been independently validated. Additionally, recent transcriptomic and epigenetic studies focusing on endocrine differentiation have identified PanNET subtypes that display similarities to either α-cells or β-cells and differ in clinical outcomes. Thus, future prospective studies that incorporate genomic and epigenetic biomarkers are warranted and have translational potential for individualized therapeutic and surveillance strategies.
胰腺神经内分泌肿瘤（PanNETs）是一种极具临床挑战性的疾病，因为这些肿瘤在临床表现、自然病史和预后方面存在差异。需要新的预后生物标志物来改善患者分层和治疗选择。已经独立验证了几种推测的预后和/或预测生物标志物（例如，端粒的非经典延长、α-地中海贫血/智力迟钝、X 连锁（ATRX）/死亡结构域相关蛋白（DAXX）丢失）。此外，最近专注于内分泌分化的转录组学和表观遗传学研究已经确定了 PanNET 亚型，这些亚型与 α 细胞或 β 细胞具有相似性，并且在临床结果上存在差异。因此，未来的前瞻性研究纳入基因组和表观遗传生物标志物是必要的，并且具有转化为个体化治疗和监测策略的潜力。
