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1型多发性内分泌腺瘤病中小肾上腺病变的自然病程:一项内镜超声成像研究。

Natural course of small adrenal lesions in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study.

作者信息

Schaefer S, Shipotko M, Meyer S, Ivan D, Klose K J, Waldmann J, Langer P, Kann P H

机构信息

Division of Endocrinology and Diabetology, Philipp's University, D-35033 Marburg, Germany (EU).

出版信息

Eur J Endocrinol. 2008 May;158(5):699-704. doi: 10.1530/EJE-07-0635.

Abstract

OBJECTIVE

Adrenal lesion is one of the features of multiple endocrine neoplasia type 1 (MEN1). This study aimed to assess prevalence, natural course and clinical relevance of small adrenal lesions without clinical symptoms, endocrine activity, or mechanical problems and thus without clear indication for surgical therapy by endoscopic ultrasound (EUS).

DESIGN AND METHODS

Forty-nine patients with familial MEN1 were studied. Twenty-seven of these with adrenal lesions were detected by EUS and at least two performed EUS examinations were included into a subgroup where changes in adrenal morphology were studied by measuring changes in the largest diameter of the dominant adrenal tumour.

RESULTS

EUS detected adrenal lesions in 36 (73%) patients: 6 (12%) plump adrenals, 17 (35%) nodular hyperplasia, 12 (24%) adenomas and 1 (2%) cyst. Bilateral adrenal lesions were detected in 17 patients and unilateral in 19 patients. A change in the largest tumour diameter was found to be for nodular hyperplasia -0.02+/-1.41% per month (range -2.56 to 4.58%) and for adenomas -0.61+/-1.95% per month (range -6.25 to 1.15%). One patient had an adrenal cyst with significant growth. There was no evidence of carcinoma or metastatic disease during the study.

CONCLUSIONS

The prevalence of adrenal lesions in MEN1 is higher than that reported earlier. Except one cystic lesion, no significant change in the tumour size was observed over a mean observation period of more than 2 years. In a typical situation, small adrenal lesions in MEN1 seem to be constant in their morphology.

摘要

目的

肾上腺病变是1型多发性内分泌腺瘤病(MEN1)的特征之一。本研究旨在评估无临床症状、内分泌活性或机械问题且因此无内镜超声(EUS)手术治疗明确指征的小肾上腺病变的患病率、自然病程及临床相关性。

设计与方法

对49例家族性MEN1患者进行研究。其中27例肾上腺病变患者通过EUS检测到,且至少进行了两次EUS检查,这些患者被纳入一个亚组,通过测量优势肾上腺肿瘤最大直径的变化来研究肾上腺形态的变化。

结果

EUS在36例(73%)患者中检测到肾上腺病变:6例(12%)肾上腺饱满,17例(35%)结节样增生,12例(24%)腺瘤,1例(2%)囊肿。17例患者检测到双侧肾上腺病变,19例患者为单侧病变。发现结节样增生的肿瘤最大直径每月变化为-0.02±1.41%(范围-2.56至4.58%),腺瘤为-0.61±1.95%(范围-6.25至1.15%)。1例患者肾上腺囊肿有显著生长。研究期间无癌或转移疾病的证据。

结论

MEN1中肾上腺病变的患病率高于先前报道。除1例囊性病变外,在平均超过2年的观察期内未观察到肿瘤大小有显著变化。在典型情况下,MEN1中的小肾上腺病变形态似乎是稳定的。

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