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近期小肠类癌神经内分泌肿瘤患者的腹腔内纤维化。

Intra-abdominal fibrosis in a recent cohort of patients with neuroendocrine ('carcinoid') tumours of the small bowel.

机构信息

Department of Endocrinology, Barts and the London School of Medicine, London, UK.

出版信息

QJM. 2010 Mar;103(3):177-85. doi: 10.1093/qjmed/hcp191. Epub 2010 Feb 1.

Abstract

BACKGROUND

Fibrosis is a hallmark of neuroendocrine tumours (NETs) arising in the jejunum and ileum and may manifest in the mesentery and elsewhere. It is clinically important and once-established, there are few effective therapies.

AIM

To examine the frequency, radiological manifestations and clinical significance of intra-abdominal fibrosis in a patient cohort using modern cross-sectional imaging. Current prevalence is compared to historical series and correlation with cardiac fibrosis evaluated.

DESIGN

Cross-sectional, retrospective survey of a cohort of patients with mid-gut NETs from a single centre.

METHODS

Review of clinical features, biochemistry and imaging of patients with sporadic mid-gut NET and available imaging between 2002 and 2008.

RESULTS

Thirty-one patients were included: 26 (83.9%) had liver metastases and 11 (35.4%) had small-bowel wall thickening; 17 patients (55%) had mesenteric involvement, with a mass, which contained coarse calcification in seven patients and fine calcification in a further two. There was soft-tissue stranding in 13 patients (plus in a further patient with no mass) and 'indrawing' of tissues in 11 patients. Two patients had a 'misty' mesentery and two had early retroperitoneal fibrosis. Mesenteric involvement was unrelated to gender and urinary 5HIAA excretion.

CONCLUSION

Intra-abdominal fibrosis can be detected radiologically in around half of patients with mid-gut NET using contemporary cross-sectional imaging. Although not statistically significant, small-bowel obstruction was seen more frequently in the group with fibrosis. There was no relationship with cardiac fibrosis. Prospective studies are needed to evaluate predictors of fibrosis onset and clinical course and determine optimal methods of prevention and treatment.

摘要

背景

纤维化是发生在空肠和回肠的神经内分泌肿瘤(NETs)的一个标志,可能在肠系膜和其他部位表现出来。它在临床上很重要,一旦形成,治疗方法就很少。

目的

使用现代横断面成像技术,检查一组患者的腹腔内纤维化的频率、影像学表现和临床意义。将当前的流行率与历史系列进行比较,并评估与心脏纤维化的相关性。

设计

对来自单一中心的一组散发性中肠 NET 患者的队列进行横断面、回顾性调查。

方法

回顾性分析 2002 年至 2008 年间 31 例散发性中肠 NET 患者的临床特征、生化和影像学表现,以及可用的影像学资料。

结果

31 例患者纳入研究:26 例(83.9%)有肝转移,11 例(35.4%)有小肠壁增厚;17 例(55%)有肠系膜受累,有肿块,其中 7 例有粗钙化,2 例有细钙化。13 例患者有软组织条索状改变(另外 1 例无肿块的患者也有这种改变),11 例患者有组织“内陷”。2 例患者有“模糊”的肠系膜,2 例患者有早期腹膜后纤维化。肠系膜受累与性别和尿 5HIAA 排泄无关。

结论

使用现代横断面成像技术,大约一半的中肠 NET 患者可以在影像学上检测到腹腔内纤维化。虽然没有统计学意义,但在有纤维化的组中更频繁地观察到小肠梗阻。与心脏纤维化无关。需要前瞻性研究来评估纤维化发病和临床过程的预测因素,并确定预防和治疗的最佳方法。

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