Wynne A G, Gharib H, Scheithauer B W, Davis D H, Freeman S L, Horvath E
Division of Endocrinology, Metabolism and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905.
Am J Med. 1992 Jan;92(1):15-24. doi: 10.1016/0002-9343(92)90009-z.
The syndrome of inappropriate thyroid-stimulating hormone (TSH) secretion, characterized by elevated serum free thyroxine and triiodothyronine levels in association with measurable serum TSH concentrations, remains an uncommon cause of hyperthyroidism that is being recognized with increasing frequency. The hyperthyroidism may be due to either neoplastic pituitary TSH secretion or selective pituitary resistance to thyroid hormone. In an effort to better understand this rare cause of hyperthyroidism, we undertook a retrospective analysis of our institution's experience with this condition.
We reviewed our cumulative experience (10 patients) with hyperthyroidism due to the syndrome of inappropriate secretion of TSH.
Six patients were diagnosed with TSH-secreting pituitary adenomas and four were found to have selective pituitary resistance to thyroid hormone. One patient with tumor had a TSH-secreting pituitary adenoma in the setting of multiple endocrine neoplasia syndrome. In all patients with tumor, hyperthyroidism was successfully treated with transsphenoidal adenomectomy with or without pituitary radiotherapy. All four patients with pituitary resistance had thyroid ablation or resection prior to their correct diagnosis. Therefore, therapy for this group of patients involved thyroid hormone replacement and efforts to suppress TSH hypersecretion. All 10 patients have done well clinically, with follow-up ranging from 2 weeks to 13 years.
Adequate treatment exists for the two primary causes of TSH hypersecretion. TSH-secreting pituitary adenomas are treated with surgery and, if necessary, adjuvant pituitary radiotherapy. The results are generally good if the tumor is diagnosed and treated at an early stage. Primary therapy for hyperthyroidism due to selective pituitary resistance to thyroid hormone is aimed at suppression of pituitary TSH hypersecretion. The evaluation of any patient with hyperthyroidism must be thorough and, in some cases, should include measurement of TSH to determine the presence of inappropriate secretion. Eliminating this diagnosis will help avoid improper and potentially harmful treatment of hyperthyroid patients.
不适当促甲状腺激素（TSH）分泌综合征的特征是血清游离甲状腺素和三碘甲状腺原氨酸水平升高，同时伴有可检测到的血清TSH浓度，它仍然是一种不太常见的甲状腺功能亢进病因，但其被识别的频率正在增加。甲状腺功能亢进可能是由于垂体肿瘤性TSH分泌或垂体对甲状腺激素的选择性抵抗所致。为了更好地理解这种罕见的甲状腺功能亢进病因，我们对本机构处理该病症的经验进行了回顾性分析。
我们回顾了因TSH分泌不当综合征导致甲状腺功能亢进的累积经验（10例患者）。
6例患者被诊断为分泌TSH的垂体腺瘤，4例被发现存在垂体对甲状腺激素的选择性抵抗。1例肿瘤患者在多发性内分泌肿瘤综合征背景下患有分泌TSH的垂体腺瘤。在所有肿瘤患者中，经蝶窦腺瘤切除术联合或不联合垂体放疗成功治疗了甲状腺功能亢进。所有4例垂体抵抗患者在正确诊断之前均接受了甲状腺消融或切除术。因此，该组患者的治疗包括甲状腺激素替代以及抑制TSH分泌过多的措施。所有10例患者临床情况良好，随访时间从2周至13年不等。
对于TSH分泌过多的两种主要病因均有充分的治疗方法。分泌TSH的垂体腺瘤采用手术治疗，必要时辅以垂体放疗。如果肿瘤在早期被诊断和治疗，结果通常良好。因垂体对甲状腺激素选择性抵抗导致的甲状腺功能亢进的主要治疗方法旨在抑制垂体TSH分泌过多。对任何甲状腺功能亢进患者的评估都必须全面，在某些情况下，应包括测定TSH以确定是否存在不适当分泌。排除该诊断有助于避免对甲状腺功能亢进患者进行不适当且可能有害的治疗。
