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嗜铬细胞瘤合并亚临床库欣综合征及垂体微腺瘤。

Phaeochromocytoma combined with subclinical Cushing's syndrome and pituitary microadenoma.

作者信息

Yaylali Guzin Fidan, Akin Fulya, Bastemir Mehmet, Yaylali Yalin Tolga, Ozden Akin

机构信息

Pamukkale University, School of Medicine, Department of Endocrinology and Metabolic Diseases, Denizli, Turkey.

出版信息

Clin Invest Med. 2008;31(3):E176-81. doi: 10.25011/cim.v31i3.3475.

Abstract

OBJECTIVES

Phaeochromocytoma (PHEO) occasionally associates with pathological lesions of the adrenal cortex. The coexistence of PHEO and pre-clinical Cushing's syndrome (PCS) of the same adrenal gland has rarely been reported. We report a case of PHEO and PCS originating from the same adrenal gland and discuss the peculiar diagnostic aspects of this entity.

CLINICAL PRESENTATION

A 64 yr old man was hospitalized to evaluate the right adrenal mass which was discovered incidentally by ultrasonography. He had a history of type 2 diabetes mellitus and hyperlipidemia. Blood pressure measurements were all normal during his hospital stay. Laboratory examination showed: urinary catecholamines were markedly increased. HbA1C of 14.3 %, midnight cortisol of 11(microg/dL), cortisol was not suppressed after the overnight 1 mg oral dexamethasone suppression test (DST): 3.42(microg/dL), 24 hr free cortisol in the urine : 213 microg/day (10-100), cortisol levels were suppressed more than 50% with 8 mg of dexamethasone. CT scan of the adrenal glands showed a 6 cm well encapsulated right adrenal mass together with a clearly normal left adrenal gland. MRI investigation of the sella turcica revealed a pituitary microadenoma on the right side of the adenohypophysis He was treated with alpha and subsequent beta blockers after the diagnosis of PHEO and PCS was made. Right adrenalectomy was performed. The pathology showed typical PHEO with adrenocortical hyperplasia. VMA, metanefrin and free cortisol levels were normalized one month after surgery.

CONCLUSION

The present report is a rare case of PHEO combined with PCS in the same adrenal gland.

摘要

目的

嗜铬细胞瘤(PHEO)偶尔会与肾上腺皮质的病理性病变相关。同一肾上腺中PHEO与临床前库欣综合征(PCS)并存的情况鲜有报道。我们报告一例起源于同一肾上腺的PHEO和PCS病例,并讨论该实体独特的诊断方面。

临床表现

一名64岁男性因超声偶然发现右肾上腺肿块而住院。他有2型糖尿病和高脂血症病史。住院期间血压测量均正常。实验室检查显示:尿儿茶酚胺明显升高。糖化血红蛋白为14.3%,午夜皮质醇为11(μg/dL),口服1mg地塞米松过夜抑制试验(DST)后皮质醇未被抑制:3.42(μg/dL),24小时尿游离皮质醇:213μg/天(10 - 100),8mg地塞米松可使皮质醇水平抑制超过50%。肾上腺CT扫描显示一个6cm边界清晰的右肾上腺肿块,左肾上腺明显正常。蝶鞍MRI检查显示腺垂体右侧有一个垂体微腺瘤。在诊断为PHEO和PCS后,他接受了α受体阻滞剂及随后的β受体阻滞剂治疗。进行了右肾上腺切除术。病理显示典型的PHEO伴肾上腺皮质增生。术后1个月,香草扁桃酸(VMA)、间甲肾上腺素和游离皮质醇水平恢复正常。

结论

本报告是同一肾上腺中PHEO合并PCS的罕见病例。

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