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一例肾上腺意外瘤发现9年后诊断为肾上腺皮质癌的病例。

A case of adrenocortical carcinoma diagnosed 9 years after the discovery of adrenal incidentaloma.

作者信息

Aono Daisuke, Kometani Mitsuhiro, Asano Takahiro, Konishi Seigo, Karashima Shigehiro, Ikeda Hiroko, Nohara Takahiro, Yoneda Takashi

机构信息

Department of Health Promotion and Medicine of the Future, Graduate School of Medical Sciences, Kanazawa University, Ishikawa 920-8641, Japan.

Medical Education Research Center, Graduate School of Medical Sciences, Kanazawa University, Ishikawa 920-8641, Japan.

出版信息

Endocr J. 2022 Dec 28;69(12):1415-1421. doi: 10.1507/endocrj.EJ22-0171. Epub 2022 Aug 6.

DOI:10.1507/endocrj.EJ22-0171
PMID:35934796
Abstract

Adrenocortical carcinoma (ACC) is an uncommon cause of adrenal incidentaloma (AI). ACCs generally occur in large sizes, >4 cm in diameter, at initial presentation and grow rapidly. Therefore, there have been few reports of cases with long-term follow-up with imaging before ACC was diagnosed. Herein, we present a case of an adrenal mass that had remained small and unchanged for 5 years but later grew rapidly and was finally diagnosed as ACC. A 77-year-old hypertensive woman was referred to our hospital for the examination of a 5.4-cm left adrenal mass. Upon reviewing her previous unenhanced computed tomography (CT) scan, a 1.6-cm and 30 Hounsfield units (HU), homogeneous, round, left adrenal mass was incidentally detected 9 years ago. This mass remained unchanged until 4 years ago. One year ago, the mass enlarged to 3.0-cm and changed into an irregular form with heterogeneous density. The hormonal evaluation during the 9 years from the discovery of the AI was inadequate. The present examination diagnosed this case as ACC with subclinical Cushing's syndrome. The patient underwent laparoscopic left adrenalectomy, and a histological diagnosis of high-grade ACC was made. The resected tumor had the CTNNB1 gene mutation. High unenhanced CT attenuation values (>10 HU) are one of the findings that raise suspicion of malignancy. This case suggests that patients with findings atypical of adenomas on an initial unenhanced CT might be carefully followed up given the possibility of development of ACCs, even if the initial tumor size is small.

摘要

肾上腺皮质癌(ACC)是肾上腺意外瘤(AI)的一种罕见病因。ACC通常在初次就诊时体积较大,直径>4 cm,且生长迅速。因此,在ACC被诊断之前,很少有关于长期影像学随访病例的报道。在此,我们报告一例肾上腺肿块病例,该肿块在5年内一直较小且无变化,但后来迅速增大,最终被诊断为ACC。一名77岁的高血压女性因检查发现左侧肾上腺有一个5.4 cm的肿块而转诊至我院。回顾她之前的非增强计算机断层扫描(CT),9年前偶然发现一个1.6 cm、30亨氏单位(HU)、均匀、圆形的左侧肾上腺肿块。该肿块直到4年前一直没有变化。1年前,肿块增大至3.0 cm,形态变为不规则,密度不均匀。从发现AI后的9年里,激素评估不充分。目前的检查将该病例诊断为伴有亚临床库欣综合征的ACC。患者接受了腹腔镜下左肾上腺切除术,组织学诊断为高级别ACC。切除的肿瘤有CTNNB1基因突变。非增强CT衰减值高(>10 HU)是引起恶性肿瘤怀疑的表现之一。该病例表明,即使初始肿瘤较小,但初次非增强CT表现不典型腺瘤的患者,鉴于有发展为ACC的可能性,也可能需要仔细随访。

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Transformation of a Benign Adrenocortical Adenoma to a Metastatic Adrenocortical Carcinoma Is Rare But It Happens.良性肾上腺皮质腺瘤转变为转移性肾上腺皮质癌虽罕见,但确有发生。
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