Watanobe H, Kudo K, Okushima T, Nakazono M, Kudo M, Takebe K
Third Department of Internal Medicine, Hirosaki University School of Medicine, Aomori, Japan.
J Endocrinol Invest. 1992 Apr;15(4):297-301. doi: 10.1007/BF03348735.
An unusual case of coexisting acromegaly and Cushing's syndrome is reported in a 34-yr-old female. There was no biochemical or morphological evidence to suggest the presence of other endocrinopathies. She did not have any family history to suggest a hereditary tendency to endocrine disorders. Her acromegaly and Cushing's syndrome were proven to be due to a pituitary somatotroph adenoma and a cortisol-producing adenoma in the right adrenocortex, respectively. Surgical removal of both tumors led to a marked biochemical improvement of the two endocrinopathies. To account for the simultaneous occurrence of the two endocrine tumors, at least two endocrine syndromes may be considered. One of them is Carney's complex. However, Cushing's syndrome in this complex is unexceptionally due to primary pigmented nodular adrenocortical disease, differing from the adrenal pathology of our patient. In addition, a lack in this case of any other characteristic suggestive of this syndrome appears to speak against this possibility. A second possibility is multiple endocrine neoplasia type 1. The absence of a parathyroid or pancreatic islet cell tumor does not strongly support this possibility, but adrenocortical lesions are not rare in this syndrome although they are only rarely functional. However, existence of similar case reports, although very few, in the literature leaves the possibility that she represents another rare variant of sporadic multiple endocrine neoplasia type I syndrome.
报道了一例34岁女性同时患有肢端肥大症和库欣综合征的罕见病例。没有生化或形态学证据表明存在其他内分泌疾病。她没有任何家族史提示有内分泌疾病的遗传倾向。她的肢端肥大症和库欣综合征经证实分别是由垂体生长激素细胞腺瘤和右侧肾上腺皮质的皮质醇分泌腺瘤引起的。手术切除这两个肿瘤使这两种内分泌疾病在生化方面有了显著改善。为了解释这两种内分泌肿瘤的同时发生，至少可以考虑两种内分泌综合征。其中之一是卡尼综合征。然而，该综合征中的库欣综合征无一例外是由原发性色素沉着性结节性肾上腺皮质疾病引起的，这与我们患者的肾上腺病理情况不同。此外，该病例缺乏提示该综合征的任何其他特征，这似乎排除了这种可能性。第二种可能性是1型多发性内分泌肿瘤。甲状旁腺或胰岛细胞瘤的缺失并不能有力地支持这种可能性，不过在该综合征中肾上腺皮质病变并不罕见，尽管它们很少有功能。然而，文献中存在类似病例报告，虽然非常少，这使得她有可能代表散发性1型多发性内分泌肿瘤综合征的另一种罕见变异型。
