肾神经内分泌肿瘤(R-NEN)是非常罕见的肿瘤,其特征在于高死亡率。
■本研究的目的是分析R-NEN患者的预后因素和治疗对总生存期的影响。
■我们在2004年至2019年的国家癌症数据库(NCDB)中确定了所有R-NEN患者,并确定了改善生存率的预后因素。
■在542个R-NEN案例中,166例(31%)为神经内分泌肿瘤1级(NET-G1),14例(3%)为神经内分泌肿瘤2级(NET-G2),169例(31%)为神经内分泌癌(NEC-NOS),18(3%)是大细胞神经内分泌癌(LC-NEC),175(32%)是小细胞神经内分泌癌(SC-NEC)。研究中所有患者的中位总生存期为44.88个月(SE,4.265;95%CI,27.57-62.19)。没有手术干预的患者的中位总生存期为7.89个月(SE0.67;95%CI,6.58-9.20),接受手术的患者的中位总生存期为136.61个月(SE16.44;95%CI,104.38-168.84,p<0.001)。年龄增加(HR,1.05;95%CI,1.03-1.06;p<0.001),T4期疾病(HR,3.17;95%CI,1.96-5.1;p<0.001),NEC-NOS组织学(HR,2.82;95%CI,1.64-4.86;p<0.001),LC-NEC组织学(HR,2.73;95%CI,1.04-7.17;p=0.041)和SC-NEC组织学(HR,5.17;95%CI,2.95-9.05;p<0.001)均为总生存期恶化的阳性预测因子。该研究的主要局限性在于其回顾性设计。
■R-NEN是一种侵袭性肿瘤,其特点是死亡率高。手术仍然是治疗的主要手段,并且已显示出对大多数患者的生存益处。
■R-NEN由几种肿瘤组织学组成,它们的侵袭性不同,其中NEC-NOS和SC-NEC最致命。手术,主要通过微创方法,是治疗的支柱,具有明显的生存益处。
UNASSIGNED: Renal neuroendocrine neoplasms (R-NEN) are exceptionally rare tumours characterized by high mortality rates.
UNASSIGNED: The objective of this study is to analyse prognostic factors and treatment impact on overall survival in patients with R-NEN.
UNASSIGNED: We identified all patients with R-NEN in the National Cancer Database (NCDB) from 2004 to 2019 and identified prognostic factors for improved survival.
UNASSIGNED: Of 542 R-NEN cases, 166 (31%) were neuroendocrine tumour grade 1 (NET-G1), 14 (3%) were neuroendocrine tumour grade 2 (NET-G2), 169 (31%) were neuroendocrine carcinoma (NEC-NOS), 18 (3%) were large cell neuroendocrine carcinoma (LC-NEC) and 175 (32%) were small cell neuroendocrine carcinoma (SC-NEC). Median overall survival for all patients in the study was 44.88 months (SE, 4.265; 95% CI, 27.57-62.19). Median overall survival was 7.89 months (SE 0.67; 95% CI, 6.58-9.20) for patients without surgical intervention and 136.61 months (SE 16.44; 95% CI, 104.38-168.84, p < 0.001) for patients who underwent surgery. Increased age (HR, 1.05; 95% CI, 1.03-1.06; p < 0.001), T4 stage disease (HR, 3.17; 95% CI, 1.96-5.1; p < 0.001), NEC-NOS histology (HR, 2.82; 95% CI, 1.64-4.86; p < 0.001), LC-NEC histology (HR, 2.73; 95% CI, 1.04-7.17; p = 0.041) and SC-NEC histology (HR, 5.17; 95% CI, 2.95-9.05; p < 0.001) were all positive predictors of worsening overall survival. The main limitation of the study is its retrospective design.
UNASSIGNED: R-NEN is an aggressive tumour characterized by high mortality rates. Surgery continues to be the mainstay of treatment and has shown to provide a survival benefit for most patients.
UNASSIGNED: R-NEN is composed of several tumour histologies that differ based on their aggressiveness with NEC-NOS and SC-NEC being the most lethal. Surgery, predominantly through minimally invasive approaches, is the mainstay of treatment and has a clear survival benefit.