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嗜酸细胞癌:一种罕见的激素分泌性肿瘤。

Oncocytic Carcinoma: A Rare Hormone-Producing Tumor.

作者信息

Guimarães Francisco, Simões Joana, Fontes Luísa, Dias Daniela Filipa, Pinheiro Nuno

机构信息

Internal Medicine, Hospital CUF (Companhia União Fabril) Descobertas, Lisbon, PRT.

Endocrinology and Diabetes, Hospital CUF (Companhia União Fabril) Descobertas, Lisbon, PRT.

出版信息

Cureus. 2025 Aug 18;17(8):e90450. doi: 10.7759/cureus.90450. eCollection 2025 Aug.

Abstract

Oncocytic adrenocortical carcinoma (OACC) is a rare type of malignancy that affects the adrenal cortex. We describe the case of a 68‑year‑old woman with months of refractory hypertension, hypokalemia, headaches, night sweats, and palpitations. Computed tomography revealed a 113 mm left adrenal mass displacing the pancreatic tail and spleen. After a multidisciplinary review, she underwent open adrenalectomy. The 355 g tumor was well circumscribed; histology showed abundant eosinophilic oncocytes arranged in nests and trabeculae, 36 mitoses per 50 high‑power fields (including atypical figures), necrosis, and venous invasion, and the Ki‑67 index was 10%. These findings satisfied major and minor Lin-Weiss-Bisceglia criteria, confirming OACC. Immunohistochemistry was positive for calretinin, Melan‑A, synaptophysin, and inhibin, consistent with adrenocortical origin. Postoperatively, hypertension and potassium levels normalized with reduced antihypertensive therapy; she commenced steroid replacement and adjuvant mitotane. At early follow‑up, she remained clinically stable and disease‑free. This case highlights the importance of considering OACC in patients with large adrenal masses and resistant hypertension, demonstrates the value of multidisciplinary management and definitive surgery, and contributes to the limited evidence guiding adjuvant therapy for this uncommon tumor.

摘要

嗜酸性肾上腺皮质癌(OACC)是一种罕见的肾上腺皮质恶性肿瘤。我们报告了一例68岁女性患者,她有数月难治性高血压、低钾血症、头痛、盗汗和心悸症状。计算机断层扫描显示左肾上腺有一个113毫米的肿块,推移了胰尾和脾脏。经过多学科会诊后,她接受了开放性肾上腺切除术。肿瘤重355克,边界清晰;组织学显示有丰富的嗜酸性嗜酸性细胞,呈巢状和小梁状排列,每50个高倍视野有36个有丝分裂(包括非典型形态)、坏死和血管侵犯,Ki-67指数为10%。这些发现符合林-魏斯-比塞利亚主要和次要标准,确诊为OACC。免疫组织化学检测钙视网膜蛋白、黑素-A、突触素和抑制素呈阳性,符合肾上腺皮质来源。术后,高血压和血钾水平通过减少降压治疗恢复正常;她开始接受类固醇替代治疗和辅助使用米托坦。在早期随访中,她临床稳定,无疾病复发。该病例强调了在肾上腺肿块大且患有难治性高血压的患者中考虑OACC的重要性,证明了多学科管理和确定性手术的价值,并为指导这种罕见肿瘤辅助治疗的有限证据提供了补充。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/77e6/12444228/7454411a87c0/cureus-0017-00000090450-i01.jpg

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本文引用的文献

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Oncocytoma of the adrenal gland medulla.肾上腺髓质嗜铬细胞瘤。
Ann Diagn Pathol. 2013 Feb;17(1):123-6. doi: 10.1016/j.anndiagpath.2011.07.002. Epub 2011 Nov 4.
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Adrenal cortical oncocytoma mimicking pheochromocytoma.肾上腺皮质嗜酸细胞瘤酷似嗜铬细胞瘤。
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