复发性肾上腺嗜铬细胞瘤致异位ACTH综合征1例并文献复习

doi:10.3969/j.issn.1004-583X.2025.09.011

摘要/Abstract

摘要：

目的探讨复发性肾上腺嗜铬细胞瘤引起异位促肾上腺皮质激素(adrenocorticotropic hormone, ACTH)综合征的临床诊断和治疗,提高临床医生对该类罕见疾病的认识及重视。方法回顾性分析1例复发性肾上腺嗜铬细胞瘤致异位ACTH综合征的病例资料,并复习相关文献。结果患者女性,64岁,因发现血糖高8年,头晕、消瘦2个月入院。既往右侧肾上腺嗜铬细胞瘤术后。患者此次顽固性低钾血症,查ACTH、皮质醇(cortisol, COR)高于检测正常值10倍以上,大、小地塞米松抑制试验不受抑制。血儿茶酚胺六项均明显升高。胸腹联合CT平扫+增强:右侧肾上腺区及右侧肾周多发占位,考虑嗜铬细胞瘤复发及肾周多发转移可能。予常规剂量酚苄明作术前准备,行腹腔镜右侧肾上腺肿瘤切除术。病理提示右侧肾上腺嗜铬细胞瘤,免疫组化CgA(+),NSE(+),Ki-67(+约2%),Sny(+),ACTH(-)。术后ACTH及皮质醇迅速降至正常范围。术后3、6、12个月复查提示儿茶酚胺及ACTH、皮质醇均处于正常范围,患者血压未再升高,血糖较前明显好转,体重逐渐恢复。结论复发性嗜铬细胞瘤引起异位ACTH综合征是一种罕见疾病,临床遇到异位ACTH综合征需考虑该可能,手术切除是有效治疗手段,术前需按照嗜铬细胞瘤完善术前准备。

关键词: 嗜铬细胞瘤, 肾上腺, 异位ACTH综合征

Abstract:

Objective To explore the clinical diagnosis and treatment of ectopic adrenocorticotropic hormone (ACTH) syndrome caused by recurrent adrenal pheochromocytoma, thus enhancing the awareness and attention of clinicians to such a rare disease. Methods Clinical data of a patient with ectopic ACTH syndrome caused by recurrent adrenal pheochromocytoma were retrospectively analyzed, and relevant literatures were reviewed. Results A 64-year-old female patient was admitted due to hyperglycemia for 8 years, accompanied by dizziness and weight loss for 2 months. She had a surgical history of right adrenal pheochromocytoma. The patient has now suffered from persistent hypokalemia. ACTH and cortisol (COR) levels were more than 10 times higher than the normal reference ranges. The high-dose and low-dose dexamethasone suppression test showed that the lease of ACTH was not suppressed. All six indicators in the blood catecholamine panel were significantly elevated. The combined plain and contrast-enhanced computed tomography (CT) scans of the chest and abdomen showed multiple occupying lesions in the right adrenal area and the right perirenal region, suggesting a possible recurrence of pheochromocytoma and multiple perirenal metastases. A conventional dose of phenoxybenzamine was administered for preoperative preparation, and laparoscopic resection of the right adrenal tumor was performed. The pathology suggested a pheochromocytoma of the right adrenal gland. The immunohistochemistry results showed CgA(+), NSE(+), Ki-67 (+ approximately 2%), Sny(+), and ACTH(-). After the surgery, ACTH and cortisol levels rapidly returned to the normal reference ranges. The follow-up examinations at 3, 6, and 12 months postoperatively indicated that catecholamines, ACTH, and cortisol were all within the normal reference ranges. The patient's blood pressure did not rise again, and the blood sugar decreased significantly compared to pre-treatment levels. The weight gradually returned to normal. Conclusion Ectopic ACTH syndrome caused by recurrent pheochromocytoma is a rare disease. Clinically, this possibility should be considered when encountering ectopic ACTH syndrome. Surgical resection is an effective treatment, and preoperative preparation should be completed in accordance with the standards for pheochromocytoma before surgery.

Key words: pheochromocytoma, adrenal gland, ectopic ACTH syndrome

中图分类号:

R736.6

解丽然, 饶小娟, 余玲, 史双伟, 方一凡, 赵明明, 焦培林, 宋淑敏, 桑艳红. 复发性肾上腺嗜铬细胞瘤致异位ACTH综合征1例并文献复习[J]. 临床荟萃, 2025, 40(9): 837-843.

Xie Liran, Rao Xiaojuan, Yu Ling, Shi Shuangwei, Fang Yifan, Zhao Mingming, Jiao Peilin, Song Shumin, Sang Yanhong. Ectopic ACTH syndrome caused by recurrent adrenal pheochromocytoma: A case report and literature review[J]. Clinical Focus, 2025, 40(9): 837-843.

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链接本文: https://www.lchc.cn/CN/10.3969/j.issn.1004-583X.2025.09.011

https://www.lchc.cn/CN/Y2025/V40/I9/837

图/表 8

表1 术前大小地塞米松试验及术后ACTH、COR激素水平

Tab.1 Preoperative high-dose and low-dose dexamethasone test and postoperative levels of ACTH and COR hormones

时间	ACTH(ng/L)			COR(nmol/L)
时间	08:00	16:00	00:00	08:00	16:00	00:00
手术前	480.5	704.0	604.2	1 248.0	1 410.0	1 214.0
基础值	793.3			1 575.0
小地塞米松	1 085.0			1 575.0
大地塞米松	1 180.0			1 547.0
手术后1 d	58.6			535.5
手术后1月(2020-05-15)	67.0	33.8	23.9	352.4	91.8	89.9
手术后1年(2021-01-22)	110.4	37.3	15.1	639.6	195.8	105.3

表2 血儿茶酚胺6项

Tab.2 Six indicators in the blood catecholamine panel

	3-甲氧酪胺 (ng/L)	甲氧基肾上腺素 (ng/L)	甲氧基去甲肾上腺素(ng/L)	多巴胺 (ng/L)	肾上腺素 (ng/L)	去甲肾上腺素
手术前(2020-03-26)	120.8	1 484.1	5 044.0	299.4	1 724.7	8 362.6
手术前(2020-04-09)	64.1	1 422.2	2 649.1	310.8	2 214.4	9 289.0
手术后3 d(2020-04-25)	16.7	68.9	224.1	53.9	113.6	1 095.9
手术后1 m(2020-05-15)	6.1	78.3	242.3	18.6	116.2	961.5
手术后1 y(2021-01-25)	20.3	29.8	300.4	10.6	41.6	927.1

图1 术前肾上腺CT平扫+增强(2020-4-8)

Fig.1 Preoperative adrenal plain and contrast-enhanced CT scans (2020-04-08)

图2 术后组织病理 a.HE400; b.HE200

Fig.2 Postoperative histopathology a. H&E, ×400; b. H&E, ×200

图3 免疫组化结果 a.CgA; b.ACTH; c.Ki-67

Fig.3 Immunohistochemical results a.CgA;b.ACTH;c.Ki-67

图4 术后3个月肾上腺CT平扫(2020-08-07)

Fig.4 Adrenal plain CT scan at 3 months postoperatively (2020-08-07)

图5 术后半年肾上腺髓质全身显像(2020-10-14)

Fig.5 Whole-body adrenal medulla imaging at 6 months postoperatively (2020-10-14)

图6 术后1年肾上腺CT(2021-01-22)

Fig.6 Adrenal CT scan at 1 year postoperatively (2021-01-22)

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