Goksu Suleyman Yasin, Ozer Muhammet, Kazmi Syed Mohammad Ali, Sanford Nina Niu, Aguilera Todd A, Ahn Chul, Hsiehchen David, Sanjeevaiah Aravind, Khosama Leticia, Bleeker Jonathan, Atiq Muslim, Beg Muhammad Shaalan
Division of Hematology and Oncology, UT Southwestern Medical Center, Dallas, TX 75390, USA.
Department of Internal Medicine, UT Southwestern Medical Center, Dallas, TX 75390, USA.
Cancers (Basel). 2020 Sep 3;12(9):2501. doi: 10.3390/cancers12092501.
We aimed to study the effect of socioeconomic differences and molecular characteristics on survival in patients with young-onset pancreatic neuroendocrine tumors (YOPNET) and typical-onset PNET (TOPNET).
We identified the patients with YOPNET (<50 years) and TOPNET (≥50 years) who underwent definitive surgery diagnosed between 2004 and 2016 using the National Cancer Database. We evaluated overall survival (OS) using the Kaplan-Meier and Cox regression methods before and after propensity score matching. A publicly available genomic dataset was used to compare mutation frequencies among the two groups.
A total of 6259 patients with PNET were included, of which 27% were YOPNET. Patients with YOPNET were more likely to be Black, Hispanic, female, and have private insurance versus patients with TOPNET (all < 0.001). Patients with YOPNET had a lower comorbidity score, but higher stage and tumor size (all < 0.001). YOPNET was associated with a greater improved OS than TOPNET before and after propensity score matching ( < 0.001). On multivariable analysis, this survival difference persisted for YOPNET as an independent prognostic factor (unmatched = 0.008; matched = 0.01). For genomic analysis, patients with YOPNET had a lower rate of multiple endocrine neoplasia type-1 (MEN-1) mutation than patients with TOPNET (26% vs. 56%, < 0.001).
YOPNET represents a disease with distinct clinical features. Patients with YOPNET who underwent definitive surgery had better OS than patients with TOPNET despite having higher stage and tumor size. YOPNET also had lower rate of MEN-1 mutation.
我们旨在研究社会经济差异和分子特征对年轻发病的胰腺神经内分泌肿瘤（YOPNET）和典型发病的胰腺神经内分泌肿瘤（TOPNET）患者生存的影响。
我们使用国家癌症数据库确定了2004年至2016年间接受根治性手术诊断的YOPNET（<50岁）和TOPNET（≥50岁）患者。我们在倾向得分匹配前后使用Kaplan-Meier和Cox回归方法评估总生存期（OS）。使用公开可用的基因组数据集比较两组之间的突变频率。
共纳入6259例PNET患者，其中27%为YOPNET。与TOPNET患者相比，YOPNET患者更可能为黑人、西班牙裔、女性且拥有私人保险（均<0.001）。YOPNET患者的合并症评分较低，但分期和肿瘤大小较高（均<0.001）。在倾向得分匹配前后，YOPNET与比TOPNET更大的OS改善相关（<0.001）。在多变量分析中，这种生存差异作为独立预后因素在YOPNET中持续存在（未匹配=0.008；匹配=0.01）。对于基因组分析，YOPNET患者的1型多发性内分泌肿瘤（MEN-1）突变率低于TOPNET患者（26%对56%，<0.001）。
YOPNET代表一种具有独特临床特征的疾病。尽管分期和肿瘤大小较高，但接受根治性手术的YOPNET患者的OS优于TOPNET患者。YOPNET的MEN-1突变率也较低。
