Lewkowicz Ewelina, Trofimiuk-Müldner Małgorzata, Wysocka Katarzyna, Pach Dorota, Kiełtyka Agnieszka, Stefańska Agnieszka, Sowa-Staszczak Anna, Tomaszewska Romana, Hubalewska-Dydejczyk Alicja
Pol Arch Med Wewn. 2015;125(5):337-46. doi: 10.20452/pamw.2832. Epub 2015 Apr 29.
Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) constitute a rare and heterogeneous group of tumors with varied biology.
The aim of this study was to establish the clinical characteristics of patients with GEP-NEN and identify factors influencing their 5-year survival.
The study included 122 patients living in Kraków or its administrative region, who were diagnosed with GEP-NEN between 2002 and 2011.
The mean follow-up period was 4.9 ±2.8 years. The most frequent primary site of the tumor was the small intestine (n = 25; 20%), followed by pancreas (n = 23; 19%), rectum (n = 23; 19%), stomach (n = 21; 17%), appendix (n = 19; 16%), and colon (n = 11; 9%). There were 84 tumors classified as NEN G1; 31, as NEN G2; 5, as neuroendocrine carcinoma; and 1, as mixed adenoneuroendocrine carcinoma. Most well-differentiated GEP-NENs (n = 57; 57%) were diagnosed at stage I according to the American Joint Committee on Cancer / Union for International Cancer Control (AJCC/UICC) classification; 77% of NEN G1 (n = 64) were diagnosed at stage I, but the majority of NEN G2—at stage IV (n = 18; 58%). Metastases at diagnosis were found in 38 patients (34%). In 90% of the cases (n = 101), tumors were hormonally nonfunctional. The overall 5-year survival was 85%. In the univariate analysis, NEN G2 (P = 0.003), higher stage according to the AJCC/UICC classification (P <0.001), and metastases at diagnosis (P <0.001) were associated with poorer prognosis. In standardized multivariate models, higher stage (P = 0.02) and metastases at diagnosis (P = 0.02) were independent risk factors for death.
The most important factors affecting survival of patients with GEP-NENs are tumor stage and the presence of metastases at diagnosis. The analysis of single-center data improves identification of patients with poorer prognosis requiring a more aggressive approach.
胃肠胰神经内分泌肿瘤(GEP-NENs)是一组罕见且异质性的肿瘤,生物学行为多样。
本研究旨在明确GEP-NEN患者的临床特征,并确定影响其5年生存率的因素。
本研究纳入了122例居住在克拉科夫或其行政区的患者,这些患者在2002年至2011年间被诊断为GEP-NEN。
平均随访期为4.9±2.8年。肿瘤最常见的原发部位是小肠(n = 25;20%),其次是胰腺(n = 23;19%)、直肠(n = 23;19%)、胃(n = 21;17%)、阑尾(n = 19;16%)和结肠(n = 11;9%)。有84个肿瘤被分类为NEN G1;31个为NEN G2;5个为神经内分泌癌;1个为混合性腺神经内分泌癌。根据美国癌症联合委员会/国际癌症控制联盟(AJCC/UICC)分类,大多数高分化GEP-NENs(n = 57;57%)在I期被诊断;77%的NEN G1(n = 64)在I期被诊断,但大多数NEN G2在IV期被诊断(n = 18;58%)。38例患者(34%)在诊断时发现有转移。在90%的病例(n = 101)中,肿瘤无激素功能。总体5年生存率为85%。在单因素分析中,NEN G2(P = 0.003)、根据AJCC/UICC分类的更高分期(P <0.001)以及诊断时的转移(P <0.001)与较差的预后相关。在标准化多变量模型中,更高分期(P = 0.02)和诊断时的转移(P = 0.02)是死亡的独立危险因素。
影响GEP-NEN患者生存的最重要因素是肿瘤分期和诊断时是否存在转移。对单中心数据的分析有助于更好地识别预后较差、需要更积极治疗方法的患者。