Notsu K, Oka N, Sohmiya M, Sato T, Ando S, Moritake K, Inada K, Osamura Y, Kato Y
Department of Medicine, Shimane Medical University, Izumo, Japan.
Endocr J. 1994 Dec;41(6):631-7. doi: 10.1507/endocrj.41.631.
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.
本文报道了一例罕见的孤立性促肾上腺皮质激素（ACTH）缺乏症，伴有抗垂体抗体、垂体囊肿、蝶窦囊肿和松果体肿瘤。一名68岁男性因全身乏力前来我院就诊。实验室检查数据显示血浆促肾上腺皮质激素（ACTH）和皮质醇水平降低，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-羟皮质类固醇（17-OHCS）水平较低，但对ACTH-Z给药有反应。其他垂体功能未受损。血清中检测到针对大鼠垂体细胞质和GH3细胞表面的抗体。磁共振成像（MRI）显示，在T1加权和T2加权图像中，垂体前叶和蝶窦有高信号强度肿块，松果体区T1加权图像中有低信号强度肿块。进行经蝶窦手术切除蝶窦和垂体中的肿块。病理研究显示蝶窦有一个良性囊肿，垂体有纤维变性和嗜碱性粒细胞减少。垂体中未检测到浸润性单核细胞。免疫组织化学研究显示垂体中产生ACTH的细胞数量减少。患者通过糖皮质激素替代治疗情况良好，可能的良性松果体肿瘤未生长。
