肾上腺嗜铬细胞瘤引起库欣综合征一例报告并文献复习

510120 广州,中山大学孙逸仙纪念医院泌尿外科 1;230000 合肥,安徽医科大学附属第二医院泌尿外科 2

肾上腺;嗜铬细胞瘤; 库欣综合征; 腹腔镜

Adrenocorticotropic hormone-secreting phaeochromocytoma: a case report and literature review
Liu Cheng1, Li Zhuohang1, Li Kuiqing1, Liu Hao1, Bi Liangkuan2, Xu Kewei1.

1Department of Urology, Sun Yat-sen memorial hospital, Sun Yat-sen University, Guangzhou, Guangdong 510120, China; 2Departments of Urology, the Second Affiliated Hospital of Anhui Medical University, Hefei 230601, China

Adrenal gland; Pheochromocytoma; Cushing's syndrome; Laparoscope

DOI: 10.3877/cma.j.issn.1674-3253.2018.03.006

备注

目的 探讨肾上腺嗜铬细胞瘤引起库欣综合征的临床诊断和治疗。方法 结合文献复习,回顾性分析 2015年 12月我院收治的 1例右侧肾上腺嗜铬细胞瘤引起库欣综合征患者的资料。患者女性,45岁,因反复头痛半年,加重伴头晕、多尿、消瘦 2个月收入我院内分泌科。术前诊断考虑为肾上腺嗜铬细胞瘤引起库欣综合征,同时按库欣综合征和嗜铬细胞瘤做术前准备,行腹膜后入路腹腔镜右侧肾上腺肿瘤切除术。结果 内分泌检查示促肾上腺皮质激素(ACTH)、皮质醇高于检测值上限,且大、小地塞米松抑制试验不受抑制。血儿茶酚胺三项均升高,以肾上腺素为主,可乐定试验不受抑制。甲氧基肾上腺素及甲氧基去甲肾上腺素均升高。胸部 CT平扫及 PET-CT未发现可疑异位ACTH来源。肾上腺 MR示双侧肾上腺内、外支弥漫性增厚,右侧肾上腺内、外侧支之间可见一类圆形肿块,边界清,考虑肾上腺嗜铬细胞瘤可能。予口服甲吡酮抑制皮质醇生成,常规剂量酚苄明作术前准备,行腹膜后入路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,合并肾上腺皮质区带性增生。免疫组化 CgA(+),ACTH(+)。术后 ACTH及皮质醇降至正常范围。术后 6个月复查提示患者高血压、糖尿病等表现消失。结论 嗜铬细胞瘤引起库欣综合征是一种罕见疾病,手术切除是有效治疗手段,术前需同时按照嗜铬细胞瘤和库欣综合征完善术前准备。
Objective To investigate the clinical diagnosis and treatment of Cushing's syndrome caused by adrenal pheochromocytoma. Methods Clinical data of one case of Cushing's syndrome caused by the right adrenal pheochromocytoma admitted to our hospital in December 2015 were retrospectively analyzed and literature review was performed. The female patient, aged 45 years old, was admitted to endocrinology department of our hospital due to recurrent headache for six months accompanied by dizziness, polyuria and weight loss for 2 months. Preoperative diagnosis prompted Cushing's syndrome caused by adrenal pheochromocytoma. Preoperative preparations were delivered according to Cushing's syndrome and pheochromocytoma. Laparoscopic resection of the right adrenal tumor was performed via the retroperitoneal approach. Results Endocrine examination prompted that adrenocorticotropic hormone (ACTH) and cortisol levels were higher than the upper limit. The large- and small-dose dexamethasone suppression tests were not inhibited. Blood catecholamines levels were all elevated, especially the adrenaline. The clonidine test was not inhibited. Both the methoxy adrenaline and methoxy norepinephrine levels were enhanced. Chest CT plain scan and PET-CT failed to identify suspected source of ectopic ACTH. Adrenal MR demonstrated diffuse thickening of the inferior and lateral branches of bilateral adrenal glands. A circular mass with clear margin was detected between the inferior and lateral branches of the right adrenal gland, which was potentially considered as adrenal pheochromocytoma. She was orally administered with metyrapone to inhibit the production of cortisol. Conventional dose of phenoxybenzamine was delivered during preoperative preparation. Laparoscopic resection of the right adrenal tumor was performed via the retroperitoneal approach. Pathological examination hinted the possibility of right adrenal pheochromocytoma accompanied with hyperplasia in the adrenal cortical zone. Immunohistochemistry revealed CgA (+) and ACTH (+). ACTH and cortisol levels were decreased to normal range after surgery. The re-examination at postoperative 6 months suggested that the symptoms of hypertension and diabetes mellitus were mitigated. Conclusion s Cushing's syndrome induced by pheochromocytoma is a rare disease. Surgical resection is an effective treatment. Full preoperative preparations should be performed according to both pheochromocytoma and Cushing's syndrome.
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