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与具有不确定恶性潜能的左肾上腺皮质嗜酸性细胞瘤相关的促肾上腺皮质激素非依赖性库欣综合征

ACTH-Independent Cushing's Syndrome Associated with Left Adrenocortical Oncocytoma of Uncertain Malignant Potential.

作者信息

Sica Giuseppe S, Siragusa Leandro, Sensi Bruno, Bellato Vittoria, Floris Pierangela, Rovella Valentina, Mauriello Alessandro, Di Prete Monia, Telesca Rossana, Ciavoni Valerio, Cardillo Carmine, Di Daniele Nicola, Tesauro Manfredi

机构信息

Department of Surgery, University of Rome "Tor Vergata", 00133 Rome, Italy.

Department of Systems Medicine, University of Rome "Tor Vergata", 00133 Rome, Italy.

出版信息

Case Rep Endocrinol. 2020 Sep 26;2020:8816527. doi: 10.1155/2020/8816527. eCollection 2020.

DOI:10.1155/2020/8816527
PMID:33062350
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7537674/
Abstract

Adrenocortical oncocytomas are rare and mostly nonfunctioning neoplasms. We report the case of a 27-year-old woman diagnosed with an ACTH-independent Cushing's syndrome due to left adrenal oncocytoma. She underwent laparoscopic adrenalectomy. Histopathological examination revealed an oncocytoma of uncertain malignant potential with a low Ki-67 proliferation index, inhibin A positivity, and chromogranin A negativity. Electron micrographs confirmed adrenal oncocytoma cells, characterized by the presence of a large amount of mitochondria. The postoperative course was uneventful, and the patient experienced a progressive regression of Cushing-related symptoms. Periodical follow-ups with MRI and cortisol dosage are required due to the neoplasm's uncertain malignant potential. Considerations on the diagnosis, pathology findings, clinical remarks, and interventions are made.

摘要

肾上腺皮质嗜酸细胞瘤罕见,大多为无功能肿瘤。我们报告一例27岁女性,因左肾上腺嗜酸细胞瘤被诊断为促肾上腺皮质激素非依赖性库欣综合征。她接受了腹腔镜肾上腺切除术。组织病理学检查显示为具有不确定恶性潜能的嗜酸细胞瘤,Ki-67增殖指数低,抑制素A阳性,嗜铬粒蛋白A阴性。电子显微镜照片证实为肾上腺嗜酸细胞瘤细胞,其特征是存在大量线粒体。术后过程顺利,患者库欣相关症状逐渐消退。由于肿瘤恶性潜能不确定,需要定期进行MRI和皮质醇剂量随访。对诊断、病理结果、临床情况及干预措施进行了讨论。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9814/7537674/2135b80d0296/CRIE2020-8816527.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9814/7537674/d3828c26fe5b/CRIE2020-8816527.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9814/7537674/650102b21e73/CRIE2020-8816527.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9814/7537674/2135b80d0296/CRIE2020-8816527.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9814/7537674/d3828c26fe5b/CRIE2020-8816527.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9814/7537674/650102b21e73/CRIE2020-8816527.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9814/7537674/2135b80d0296/CRIE2020-8816527.003.jpg

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