Neuroendocrine Tumour Unit, Centre for Gastroenterology, ENETS Centre of Excellence, Royal Free London NHS Foundation Trust, London, United Kingdom.
Department of Infectious Disease Epidemiology, School of Public Health, Imperial College London, London, United Kingdom.
Neuroendocrinology. 2018;106(4):366-380. doi: 10.1159/000486097. Epub 2018 Jan 10.
Small intestinal neuroendocrine tumours (SI NETs) represent 30-50% of small bowel neoplasms and are often associated with diverse fibrotic complications. Mesenteric fibrosis is a hallmark of SI NETs which may cause substantial morbidity and is considered an adverse feature. However, survival analyses in this group of patients are lacking.
The aim of this retrospective study was to determine the overall survival (OS) and factors affecting prognosis in a large cohort of 147 patients with SI NETs and radiological evidence of mesenteric desmoplasia from our centre. The severity of desmoplasia was graded radiologically and its effect on OS and long-term complications was assessed. The median follow-up period was 82 months.
The median OS was 8.7 years (95% CI 6.8-9.9) with an overall 5-year survival of 71%. The univariate analysis demonstrated that an age >65 years, a liver tumour burden >50% of the hepatic parenchyma, carcinoid heart disease, chromogranin A levels >10 times the upper limit of normal, and urinary 5-hydroxyindoleacetic acid (5-HIAA) levels >5 times the upper limit of normal were poor prognosticators, while primary resection was associated with a longer OS. However, only an age >65 years and urinary 5-HIAA levels >10 times the upper limit of normal remained statistically significant after multivariate analysis. The severity of mesenteric desmoplasia did not seem to demonstrate a statistically significant relationship to OS or long-term outcomes.
This study is the first comprehensive survival analysis of patients with SI NETs associated with mesenteric desmoplasia and has provided important and clinically relevant epidemiological data for this group of patients.
小肠类癌肿瘤（SI NETs）占小肠肿瘤的 30-50%，常伴有多种纤维化并发症。肠系膜纤维化是 SI NETs 的一个标志，可能导致严重的发病率，并被认为是一个不利特征。然而，目前缺乏对这组患者的生存分析。
本回顾性研究的目的是确定来自我们中心的 147 名 SI NETs 患者的总体生存率（OS）和影响预后的因素，这些患者有影像学证据表明存在肠系膜纤维变性。肠系膜纤维变性的严重程度通过影像学分级，并评估其对 OS 和长期并发症的影响。中位随访时间为 82 个月。
中位 OS 为 8.7 年（95%CI 6.8-9.9），总体 5 年生存率为 71%。单因素分析表明，年龄>65 岁、肝脏肿瘤负荷>50%的肝实质、类癌性心脏病、嗜铬粒蛋白 A 水平>正常值的 10 倍、尿 5-羟吲哚乙酸（5-HIAA）水平>正常值的 5 倍是预后不良的因素，而原发切除与更长的 OS 相关。然而，只有年龄>65 岁和尿 5-HIAA 水平>正常值的 10 倍在多因素分析后仍然具有统计学意义。肠系膜纤维变性的严重程度似乎与 OS 或长期结果没有统计学上的显著关系。
本研究是首例对伴有肠系膜纤维变性的 SI NETs 患者进行的全面生存分析，为这组患者提供了重要的、有临床意义的流行病学数据。
