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家族性腺瘤性息肉病患者的肾上腺肿块。

Adrenal masses in patients with familial adenomatous polyposis.

作者信息

Marchesa P, Fazio V W, Church J M, McGannon E

机构信息

Department of Colorectal Surgery, The Cleveland Clinic Foundation, Ohio 44195, USA.

出版信息

Dis Colon Rectum. 1997 Sep;40(9):1023-8. doi: 10.1007/BF02050923.

Abstract

PURPOSE

The aims of this study were 1) to report the characteristics and the clinical outcome of familial adenomatous polyposis (FAP) patients with adrenal masses in the FAP registry at the Cleveland Clinic Foundation and 2) to estimate the prevalence of adrenal masses detected by computed tomography in FAP patients compared with that expected in a normal population.

METHODS

A retrospective review was undertaken of the FAP registry database at our institution. Only 738 patients treated at the Cleveland Clinic Foundation were included in the study. A meta-analysis was conducted to determine the relative risk of adrenal incidentaloma in this series of FAP patients and in a general population as reported in the four largest pertinent studies published in the past 15 years.

RESULTS

Fifteen patients (11 females) were identified. Two patients had symptoms related to cortisol hypersecretion (arterial hypertension) and underwent surgery. The final pathology was adrenocortical carcinoma and bilateral nodular hyperplasia. Adrenal masses were found incidentally (incidentalomas) in 13 patients: 12 were detected by computed tomography and one during laparotomy for total abdominal colectomy. Only one patient underwent left adrenalectomy for a 5-cm mass. Pathologic report revealed adrenocortical adenoma. Among the 738 patients considered in this study, only 162 underwent abdominal computed tomographic scan, mainly for assessing intra-abdominal desmoid. The prevalence of incidentaloma in our series compared with that reported in the literature is significantly different (7.4 vs. 0.6-3.4 percent; P < 0.001 (chi-squared test)).

DISCUSSION

Although the presence of other extracolonic manifestations represents a selection bias for computed tomographic study in our series, the incidence of incidentalomas in FAP patients seems to be higher than in a general population. However, incidental detection of an adrenal mass in FAP patients has probably a limited clinical relevance, and the management should be the same as that for the normal population.

摘要

目的

本研究的目的是:1)报告克利夫兰诊所基金会家族性腺瘤性息肉病(FAP)登记处中患有肾上腺肿块的FAP患者的特征和临床结果;2)估计FAP患者中通过计算机断层扫描检测到的肾上腺肿块的患病率,并与正常人群中的预期患病率进行比较。

方法

对我们机构的FAP登记数据库进行了回顾性研究。本研究仅纳入了在克利夫兰诊所基金会接受治疗的738例患者。进行了一项荟萃分析,以确定这一系列FAP患者和过去15年发表的四项最大的相关研究中报道的普通人群中肾上腺偶发瘤的相对风险。

结果

共识别出15例患者(11例女性)。2例患者有与皮质醇分泌过多相关的症状(动脉高血压)并接受了手术。最终病理结果为肾上腺皮质癌和双侧结节性增生。13例患者的肾上腺肿块是偶然发现的(偶发瘤):12例通过计算机断层扫描检测到,1例在全腹结肠切除术的剖腹手术中发现。只有1例患者因5厘米的肿块接受了左肾上腺切除术。病理报告显示为肾上腺皮质腺瘤。在本研究纳入的738例患者中,只有162例接受了腹部计算机断层扫描,主要用于评估腹内硬纤维瘤。我们系列中偶发瘤的患病率与文献报道的患病率有显著差异(7.4%对0.6%-3.4%;P<0.001(卡方检验))。

讨论

尽管其他结肠外表现的存在代表了我们系列中计算机断层扫描研究的选择偏倚,但FAP患者中偶发瘤的发生率似乎高于普通人群。然而,FAP患者中肾上腺肿块的偶然发现可能临床相关性有限,其管理应与正常人群相同。

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