Department of Hematology & Oncology, School of Medicine, Pontificia Universidad Católica de Chile, Diagonal Paraguay 362, 6th floor, Santiago, Chile.
Department of Internal Medicine, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
Horm Cancer. 2019 Feb;10(1):3-10. doi: 10.1007/s12672-018-0354-5. Epub 2018 Nov 22.
Neuroendocrine tumors (NETs) are relatively rare and highly heterogeneous neoplasms. Despite this, recent studies from North America and Central Europe have suggested an increase in incidence. In Latin America, NET data are scarce and scattered with only a few studies reporting registries. Our goal was to establish a NET registry in Chile. Here, we report the establishment and our first 166 NET patients. We observed a slight preponderance of males, a median age at diagnosis of 53 years and a median overall survival of 110 months. As anticipated, most tumors were gastroenteropancreatic (GEP). Survival analyses demonstrated that non-GEP or stage IV tumors presented significantly lower overall survival (OS). Similarly, patients with surgery classified as R0 had better OS compared to R1, R2, or no surgery. Furthermore, patients with elevated chromogranin A (CgA) or high Ki67 showed a trend to poorer OS; however, these differences did not reach statistical significance (log-rank test p = 0.07). To the best of our knowledge, this is the first report of a NET registry in Chile. Median OS in our registry (110 months) is in line with other registries from Argentina and Spain. Other variables including age at diagnosis and gender were similar to previous studies; however, our data indicate a high proportion of small-bowel NETs compared to other cohorts, reflecting the need for NET regional registries. Indeed, these registries may explain regional discrepancies in incidence and distribution, adding to our knowledge on this seemingly rare, highly heterogeneous disease.
神经内分泌肿瘤(NETs)是相对罕见且高度异质性的肿瘤。尽管如此,来自北美和中欧的最近研究表明发病率有所增加。在拉丁美洲,NET 数据稀缺且分散,只有少数研究报告了登记处。我们的目标是在智利建立一个 NET 登记处。在这里,我们报告了该登记处的建立情况以及我们的前 166 名 NET 患者。我们观察到男性略占优势,诊断时的中位年龄为 53 岁,中位总生存期为 110 个月。正如预期的那样,大多数肿瘤是胃肠胰神经内分泌肿瘤(GEP)。生存分析表明,非 GEP 或 IV 期肿瘤的总体生存率(OS)显著降低。同样,手术分类为 R0 的患者的 OS 明显优于 R1、R2 或无手术的患者。此外,CgA 升高或 Ki67 高的患者 OS 较差的趋势;然而,这些差异没有达到统计学意义(对数秩检验 p=0.07)。据我们所知,这是智利首个 NET 登记处的报告。我们的登记处的中位 OS(110 个月)与来自阿根廷和西班牙的其他登记处一致。其他变量,包括诊断时的年龄和性别,与以前的研究相似;然而,我们的数据表明,与其他队列相比,我们的小肠 NET 比例较高,这反映了需要建立 NET 区域登记处。事实上,这些登记处可能会解释发病率和分布的区域差异,增加我们对这种看似罕见、高度异质性疾病的认识。