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以低血糖为表现的纯雄激素分泌性肾上腺皮质癌

Pure Androgen-Secreting Adrenocortical Carcinoma Presenting with Hypoglycemia.

作者信息

Karimi Fariba, Dehghanian Amirreza, Fallahi Mohammadjavad, Dalfardi Behnam

机构信息

Endocrinology and Metabolism Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Trauma Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Arch Iran Med. 2019 Sep 1;22(9):527-530.

PMID:31679375
Abstract

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Most patients present with steroid hormone excess or abdominal mass effect. Pure androgen-secreting ACCs are rare, while hypoglycemia is an unusual presentation of this malignancy. We present a 26-year-old woman with hypoglycemia and history of adrenalectomy due to a large adrenal mass which was diagnosed as nonfunctional adrenal adenoma. She was admitted in our hospital 10 days after her fetal loss with repeated episodes of severe hypoglycemia. She had a high serum dehydroepiandrosterone sulfate (DHEA-S) and her hypoglycemia was associated with low insulin and C-peptide levels. Imaging revealed liver metastasis and immunohistochemical studies of the biopsied lesions confirmed the diagnosis of ACC.

摘要

肾上腺皮质癌(ACC)是一种罕见的侵袭性恶性肿瘤。大多数患者表现为类固醇激素分泌过多或腹部肿块效应。单纯分泌雄激素的ACC较为罕见,而低血糖是这种恶性肿瘤的一种不寻常表现。我们报告一名26岁女性,因巨大肾上腺肿块行肾上腺切除术,术后有低血糖病史,该肿块曾被诊断为无功能肾上腺腺瘤。她在流产后10天因反复出现严重低血糖入住我院。她的血清硫酸脱氢表雄酮(DHEA-S)水平较高,且低血糖与胰岛素和C肽水平降低有关。影像学检查发现肝转移,活检病变的免疫组化研究证实为ACC。

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引用本文的文献

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Adrenal cortical carcinoma with non‑islet cell tumor hypoglycemia: A case report.肾上腺皮质癌伴非胰岛细胞瘤性低血糖症:一例报告
Oncol Lett. 2025 Mar 7;29(5):225. doi: 10.3892/ol.2025.14971. eCollection 2025 May.
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Pure androgen-secreting adrenal tumor (PASAT): A rare case report of bilateral PASATs and a systematic review.单纯雄激素分泌性肾上腺肿瘤(PASAT):双侧 PASAT 罕见病例报告及系统评价。
Front Endocrinol (Lausanne). 2023 Mar 22;14:1138114. doi: 10.3389/fendo.2023.1138114. eCollection 2023.
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Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging.
肾上腺皮质癌:世界卫生组织重新分类及病理分期后临床与病理特征的更新
Biomedicines. 2021 Feb 10;9(2):175. doi: 10.3390/biomedicines9020175.