Kageyama Kazunori, Moriyama Takako, Sakihara Satoru, Kawashima Shoko, Suda Toshihiro
The Third Department of Internal Medicine, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori 036-8562, Japan.
Endocr J. 2003 Jun;50(3):325-31. doi: 10.1507/endocrj.50.325.
A 75-year-old woman had tumors in her pituitary, thyroid and left adrenal gland. Plasma ACTH and cortisol levels were both mildly elevated. Both plasma ACTH and cortisol concentrations were partially suppressed by 1 mg of overnight dexamethasone suppression test, while both were inhibited with a dosage of 8 mg dexamethasone. Plasma ACTH and cortisol levels were increased in response to human CRH and desmopressin. Together with the observation of pituitary microadenoma, the patient had a pituitary ACTH-producing tumor. The patient, however, had no typical Cushingoid features, hypertension, or impaired glucose tolerance, suggesting that the tumor had an autonomic ACTH secretion that was insufficient for expressing clinical symptoms, the so-called preclinical Cushing's disease. A case of preclinical Cushing's disease is extremely rare. Further, the patient had thyroid papillary carcinoma and non-functioning adrenal tumor. Molecular genetic analysis demonstrated a polymorphism of the menin gene in the patient. Even without Cushingoid features in pituitary incidentaloma, we concluded that the elevated ACTH and cortisol levels should be followed up by CRH, desmopressin and dexamethasone suppression tests. This patient with preclinical Cushing's disease would be observed whether the physical conditions in the patient develop to overt Cushing's disease.
一名75岁女性的垂体、甲状腺和左肾上腺出现肿瘤。血浆促肾上腺皮质激素（ACTH）和皮质醇水平均轻度升高。过夜地塞米松抑制试验中，1毫克地塞米松可使血浆ACTH和皮质醇浓度部分受到抑制，而8毫克地塞米松则可抑制二者。静脉注射促肾上腺皮质激素释放激素（CRH）和去氨加压素后，血浆ACTH和皮质醇水平升高。结合垂体微腺瘤的观察结果，该患者患有分泌ACTH的垂体肿瘤。然而，患者并无典型的库欣样特征、高血压或糖耐量受损，提示肿瘤自主分泌的ACTH不足以引发临床症状，即所谓的临床前库欣病。临床前库欣病病例极为罕见。此外，患者还患有甲状腺乳头状癌和无功能肾上腺肿瘤。分子遗传学分析显示该患者的Menin基因存在多态性。即便垂体偶发瘤患者没有库欣样特征，我们仍得出结论，应通过CRH、去氨加压素和地塞米松抑制试验对升高的ACTH和皮质醇水平进行随访。对于该临床前库欣病患者，需观察其身体状况是否会发展为显性库欣病。
