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破裂的分泌三种激素的肾上腺皮质癌伴醛固酮增多症、皮质醇增多症和去甲肾上腺素水平升高:病例报告

Ruptured triple hormone-secreting adrenal cortical carcinoma with hyperaldosteronism, hypercortisolism, and elevated normetanephrine: a case report.

作者信息

Woo Sin Yung, Park Seongji, Kwon Kun Young, Lim Dong-Mee, Park Keun-Young, Kim Jong-Dai

机构信息

Division of Endocrinology, Department of Internal Medicine, Konyang University College of Medicine, Daejeon, Korea.

出版信息

J Yeungnam Med Sci. 2024 Oct;41(4):306-311. doi: 10.12701/jyms.2024.00626. Epub 2024 Sep 6.

Abstract

We report a case of a ruptured triple hormone-secreting adrenal mass with hyperaldosteronism, hypercortisolism, and elevated normetanephrine levels, diagnosed as adrenal cortical carcinoma (ACC) by histology. A 53-year-old male patient who initially presented with abdominal pain was referred to our hospital for angiocoagulation of an adrenal mass rupture. Abdominal computed tomography revealed a heterogeneous 19×11×15 cm right adrenal mass with invasion into the right lobe of the liver, inferior vena cava, retrocaval lymph nodes, and aortocaval lymph nodes. Angiocoagulation was performed. Laboratory evaluation revealed excess cortisol via a positive 1-mg overnight dexamethasone suppression test, primary hyperaldosteronism via a positive saline infusion test, and plasma normetanephrine levels three times higher than normal. An adrenal mass biopsy was performed for pathological confirmation to commence palliative chemotherapy because surgical management was not deemed appropriate considering the extent of the tumor. Pathological examination revealed stage T4N1M1 ACC. The patient started the first cycle of adjuvant mitotane therapy along with adjuvant treatment with doxorubicin, cisplatin, and etoposide, and was discharged. Clinical cases of dual cortisol- and aldosterone-secreting ACCs or ACCs presenting as pheochromocytomas have occasionally been reported; however, both are rare. Moreover, to the best of our knowledge, a triple hormone-secreting ACC has not yet been reported. Here, we report a rare case and its management. This case report underscores the necessity of performing comprehensive clinical and biochemical hormone evaluations in patients with adrenal masses because ACC can present with multiple hormone elevations.

摘要

我们报告一例分泌三种激素的肾上腺肿块破裂病例,伴有醛固酮增多症、皮质醇增多症和去甲肾上腺素水平升高,经组织学诊断为肾上腺皮质癌(ACC)。一名53岁男性患者最初因腹痛就诊,因肾上腺肿块破裂而被转诊至我院进行血管内凝血治疗。腹部计算机断层扫描显示右肾上腺有一个大小为19×11×15 cm的不均匀肿块,侵犯了肝脏右叶、下腔静脉、腔后淋巴结和主动脉腔淋巴结。进行了血管内凝血治疗。实验室检查显示,1毫克过夜地塞米松抑制试验阳性提示皮质醇过量,生理盐水输注试验阳性提示原发性醛固酮增多症,血浆去甲肾上腺素水平比正常高两倍。由于考虑到肿瘤范围,手术治疗不合适,因此进行了肾上腺肿块活检以进行病理确诊,从而开始姑息化疗。病理检查显示为T4N1M1期ACC。患者开始了第一周期的米托坦辅助治疗,并联合阿霉素、顺铂和依托泊苷进行辅助治疗,随后出院。偶尔有分泌皮质醇和醛固酮的ACC或表现为嗜铬细胞瘤的ACC的临床病例报道;然而,这两种情况都很罕见。此外,据我们所知,尚未有分泌三种激素的ACC的报道。在此,我们报告一例罕见病例及其治疗情况。本病例报告强调了对肾上腺肿块患者进行全面临床和生化激素评估的必要性,因为ACC可能表现为多种激素升高。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d84/11534413/dfe6724ec0a7/jyms-2024-00626f1.jpg

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