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(衰减型)家族性腺瘤性息肉病和MUTYH相关息肉病患者的肾上腺病变

Adrenal Lesions in Patients With (Attenuated) Familial Adenomatous Polyposis and MUTYH-Associated Polyposis.

作者信息

Kallenberg Frank G J, Bastiaansen Barbara A J, Nio C Yung, Soeters Maarten R, Boermeester Marja A, Aalfs Cora M, Bossuyt Patrick M M, Dekker Evelien

机构信息

1 Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 2 Department of Radiology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 3 Department of Endocrinology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 4 Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 5 Department of Clinical Genetics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 6 Department of Clinical Epidemiology, Biostatistics, and Bioinformatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.

出版信息

Dis Colon Rectum. 2017 Oct;60(10):1057-1064. doi: 10.1097/DCR.0000000000000809.

DOI:10.1097/DCR.0000000000000809
PMID:28891849
Abstract

BACKGROUND

The reported proportion of patients with familial adenomatous polyposis who have adrenal lesions varies between 7% and 13% compared with 4% in the general population; the prevalence of adrenal lesions in patients with attenuated familial adenomatous polyposis and MUTYH-associated polyposis is unknown. Data on the clinical relevance and clinical course are limited.

OBJECTIVE

We aimed to report on the frequency, characteristics, and progression of adrenal lesions in polyposis patients.

DESIGN

This was a historical cohort study.

SETTINGS

The study was performed at the Academic Medical Center, Amsterdam.

PATIENTS

All of the patients with familial adenomatous polyposis, attenuated familial adenomatous polyposis, and MUTYH-associated polyposis were included. Medical charts and imaging reports were analyzed for data on adrenal lesions. A radiologist reassessed all of the images. Patients had not routinely been screened for adrenal lesions.

MAIN OUTCOME MEASURES

The frequency, characteristics, and progression of adrenal lesions in patients with polyposis who underwent abdominal imaging were assessed. Findings were compared with a reference.

RESULTS

A total of 39 adrenal lesions were identified in 23 (26%) of 90 patients with familial adenomatous polyposis, 2 (18%) of 11 with attenuated familial adenomatous polyposis, and 5 (24%) of 21 with MUTYH-associated polyposis. Mean age at time of detection was 50.7 years (range, 17.1-83.3 y). Median lesion size at baseline was 1.4 cm (range, 1.0-5.0 cm) versus 1.7 cm (range, 1.0-5.7 cm) after a median of 3.5 years (range, 1.0-11.4 y). Two patients were diagnosed with a hyperfunctioning lesion, and 4 underwent adrenalectomy: 3 lesions appeared benign, and 1 was oncocytic of uncertain malignant potential. The OR for detecting at least 1 lesion in a patient with polyposis versus reference was 6.2 (95% CI, 3.2-12.3), with no significant differences in ORs among the 3 syndromes.

LIMITATIONS

The study was limited by its retrospective design.

CONCLUSIONS

Adrenal lesions are frequent in patients with polyposis who undergo abdominal imaging. They appear to follow a benign and slowly progressive course and are mostly nonhyperfunctioning. See Abstract Video at http://links.lww.com/DCR/A323.

摘要

背景

据报道,家族性腺瘤性息肉病患者中肾上腺病变的比例在7%至13%之间,而普通人群中的这一比例为4%;轻度家族性腺瘤性息肉病和MUTYH相关息肉病患者中肾上腺病变的患病率尚不清楚。关于其临床相关性和临床病程的数据有限。

目的

我们旨在报告息肉病患者肾上腺病变的发生率、特征和进展情况。

设计

这是一项历史性队列研究。

地点

该研究在阿姆斯特丹学术医疗中心进行。

患者

纳入所有家族性腺瘤性息肉病、轻度家族性腺瘤性息肉病和MUTYH相关息肉病患者。分析病历和影像学报告以获取肾上腺病变的数据。一名放射科医生重新评估了所有图像。患者此前未常规筛查肾上腺病变。

主要观察指标

评估接受腹部影像学检查的息肉病患者肾上腺病变的发生率、特征和进展情况。将结果与对照组进行比较。

结果

在90例家族性腺瘤性息肉病患者中的23例(26%)、11例轻度家族性腺瘤性息肉病患者中的2例(18%)以及21例MUTYH相关息肉病患者中的5例(24%)中,共发现39处肾上腺病变。检测时的平均年龄为50.7岁(范围17.1 - 83.3岁)。基线时病变的中位大小为1.4 cm(范围1.0 - 5.0 cm),经过中位3.5年(范围1.0 - 11.4年)后为1.7 cm(范围1.0 - 5.7 cm)。2例患者被诊断为功能亢进性病变,4例接受了肾上腺切除术:3处病变表现为良性,1处为具有不确定恶性潜能的嗜酸性细胞瘤。息肉病患者与对照组相比,检测到至少1处病变的比值比为6.2(95%可信区间,3.2 - 12.3),3种综合征之间的比值比无显著差异。

局限性

本研究受其回顾性设计的限制。

结论

接受腹部影像学检查的息肉病患者中肾上腺病变很常见。它们似乎呈良性且进展缓慢,大多无功能亢进。见http://links.lww.com/DCR/A323的摘要视频。

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