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[1例肾上腺嗜铬细胞瘤与肾上腺皮质腺瘤混合型肿瘤伴发糖尿病和高血压]

[A case of adrenal mixed tumor of pheochromocytoma and adrenocortical adenoma presenting diabetes mellitus and hypertension].

作者信息

Akai H, Sanoyama K, Namai K, Miura Y, Murakami O, Hanew K, Sasaki H, Kimura N, Takahashi K, Sasano N

机构信息

Third Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan.

出版信息

Nihon Naibunpi Gakkai Zasshi. 1993 Aug 20;69(7):659-69. doi: 10.1507/endocrine1927.69.7_659.

Abstract

A 61-year-old woman with hyper-catecholaminemia and hyper-glucocorticoidemia due to a mixed tumor of the right adrenal gland is described. The patient, who had been medicated for hypertension since 1977, complained of thirst and general malaise in 1986. Body weight loss was remarkable. There was neither absolute truncal obesity nor moon face. In September 1986, her blood pressure was 180/110 mmHg and blood glucose level was 400mg/dl. Noradrenaline levels in plasma and in urine were remarkably elevated (1659 pg/ml and 120 micrograms/day, respectively), and adrenaline levels were also high (397 pg/ml in plasma, 34 micrograms/day in urine). Plasma cortisol and urinary 17-OHCS were elevated (39.2 micrograms/dl and 11.9 mg/day, respectively). Plasma ACTH was in the normal range (42.6 pg/ml). Oral administration of neither 1mg nor 8 mg of dexamethasone suppressed plasma cortisol or ACTH levels. Both 131I-metaiodobenzylguanidine and 131I-adosterol accumulated in the right adrenal gland. In 1987 the adrenal tumor (3.0 x 3.5 cm, 30 g) was resected. After the operation, her blood pressure and blood glucose level returned to normal, so that the medication became unnecessary. Histologically it was revealed that the tumor was a mixed adenoma consisting of adreno-medullary and cortical cells (corticomedullary adenoma). The literature on 21 cases of pheochromocytoma associated with Cushing's syndrome was briefly reviewed. Mathison (1969) reported the first case of a mixed tumor of adreno-medullary and cortical cells. So far as we know the present case is the second.

摘要

本文描述了一名61岁女性,因右肾上腺混合瘤导致高儿茶酚胺血症和高糖皮质激素血症。该患者自1977年起接受高血压治疗,1986年出现口渴和全身不适。体重显著减轻。既无绝对的躯干肥胖,也无满月脸。1986年9月,她的血压为180/110 mmHg,血糖水平为400mg/dl。血浆和尿液中的去甲肾上腺素水平显著升高(分别为1659 pg/ml和120微克/天),肾上腺素水平也很高(血浆中为397 pg/ml,尿液中为34微克/天)。血浆皮质醇和尿17-羟皮质类固醇升高(分别为39.2微克/分升和11.9毫克/天)。血浆促肾上腺皮质激素在正常范围内(42.6 pg/ml)。口服1毫克和8毫克地塞米松均未抑制血浆皮质醇或促肾上腺皮质激素水平。131I-间碘苄胍和131I-胆固醇均在右肾上腺中聚集。1987年切除肾上腺肿瘤(3.0×3.5厘米,30克)。术后,她的血压和血糖水平恢复正常,因此无需再用药。组织学检查显示肿瘤为肾上腺髓质和皮质细胞组成的混合腺瘤(肾上腺髓质皮质腺瘤)。本文简要回顾了21例嗜铬细胞瘤合并库欣综合征的文献。马西森(1969年)报道了第一例肾上腺髓质和皮质细胞混合瘤。据我们所知,本病例是第二例。

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