Isolated adrenocorticotrophin deficiency associated with anti-pituitary antibodies, pituitary cyst, sphenoidal cyst and pineal tumor.
Isolated adrenocorticotrophin deficiency associated with anti-pituitary antibodies, pituitary cyst, sphenoidal cyst and pineal tumor.
复制标题
与抗垂体抗体、垂体囊肿、蝶骨囊肿和松果体瘤相关的孤立性促肾上腺皮质激素缺乏症。
作者：
K. Notsu;N. Oka;M. Sohmiya;T. Sato;S. Ando;K. Moritake;K. Inada;Y. Osamura;Y. Kato
本文报告一例罕见的孤立性ACTH缺乏症，并伴有抗垂体抗体、垂体囊肿、蝶骨囊肿和松果体肿瘤。一位68岁男性因全身疲劳就诊于我们诊所。实验室数据显示，血浆促肾上腺皮质激素(ACTH)和皮质醇水平低，对胰岛素诱导的低血糖和促肾上腺皮质激素释放因子(CRF)反应迟钝。尿17-uchS较低，但对ACTH-Z有反应。其他垂体腺功能均未受损。血清中可检测到抗大鼠垂体细胞质和GH3细胞表面抗体。磁共振成像(MRI)在T1和T2加权像上均显示垂体前叶和蝶窦的高信号肿块，在松果体区的T1加权像上显示低信号肿块。采用经蝶手术切除蝶窦和垂体内的肿块。病理检查显示蝶窦内有良性囊肿，垂体部纤维变性，嗜碱性粒细胞减少。垂体内未检测到浸润性单个核细胞。免疫组织化学研究显示，垂体中产生ACTH的细胞数量减少。患者接受糖皮质激素替代治疗后维持良好，无任何可能良性松果体肿瘤的生长。
This paper reports a rare case of isolated ACTH deficiency associated with anti-pituitary antibodies, pituitary cyst, sphenoidal cyst and pineal tumor. A 68-year-old man consulted our clinic for general fatigue. Laboratory data showed low plasma adrenocorticotrophin (ACTH) and cortisol levels with blunted responses to insulin-induced hypoglycemia and corticotrophin releasing factor (CRF). Urinary 17-OHCS was low but responded to ACTH-Z administration. No other pituitary functions were impaired. Antibodies to the cytoplasm of rat pituitary and the surface of GH3 cells were detected in the serum. The magnetic resonance imaging (MRI) showed a high signal intensity mass in the anterior pituitary and in the sphenoidal sinus in both T1 and T2 weighted images as well as a low signal intensity mass in a T1 weighted image of the pineal region. Transsphenoidal surgery was performed to resect the mass in the sphenoid sinus and in the pituitary. Pathological studies showed a benign cyst in the sphenoid sinus, and fibrous degeneration and decreased basophils in the pituitary. No infiltrative mononuclear cells were detected in the pituitary. Immunohistochemical studies revealed a decrease in the number of ACTH-producing cells in the pituitary. The patient was well maintained by glucocorticoid replacement without any growth of a possibly benign pineal tumor.