垂体GH/PRL/TSH混合腺瘤四例临床特征分析及文献复习

Four patients with pituitary GH/PRL/TSH mixed adenoma:case studies and literature review

目的总结4例垂体生长激素/催乳素/促甲状腺素(GH/PRL/TSH)混合腺瘤患者的临床特征,分析其特点,探讨规范的综合治疗和管理方法。方法回顾性分析北京协和医院确诊的4例垂体GH/PRL/TSH混合腺瘤患者的临床表现、生化指标、影像学特点、治疗及转归,并进行文献复习。结果4例患者中,3例男性,起病年龄15~38岁,均以面容改变起病,3例有明显的视力下降或视野缺损;4例患者GH及胰岛素样生长因子Ⅰ(IGF-Ⅰ)明显升高,葡萄糖GH抑制试验GH谷值≥1.0 ng/mL,PRL均>100 ng/mL,三碘甲状腺原氨酸(T_(3))、甲状腺素(T_(4))升高,TSH不被抑制;4例均为大腺瘤或巨大腺瘤,侵袭性生长,1例发生垂体卒中;除1例患者因经济原因未在本院接受治疗,其余3例均接受了药物联合经鼻蝶窦垂体瘤切除术,其中1例患者中枢性甲状腺功能亢进症和高催乳素血症缓解,但GH/IGF-Ⅰ未缓解,另2例患者均≥2个激素轴未缓解。结论垂体GH/PRL/TSH混合腺瘤患者主要以面容改变为突出表现,存在多个激素轴功能亢进,腺瘤体积大,手术联合药物治疗后生化缓解率低,临床预后较差。

Objective To summarize the clinical characteristics of 4 cases of mixed pituitary adenomas involving growth hormone(GH),prolactin(PRL),and thyroid stimulating hormone(TSH),and explore the standardized management approaches.Methods The clinical data of four GH/PRL/TSH mixed pituitary adenoma patients diagnosed by Peking Union Medical College Hospital were retrospectively analyzed,including clinical manifestations,biochemical parameters,radiographic characteristics,as well as treatment and prognosis.Then literature review was conducted.Results Among the 4 patients,3 were male,with onset ages ranging from 15 to 38 years.All patients presented with coarse facial features as initial symptom.Three patients had visual impairment or visual field defects.All 4 patients had significantly elevated levels of GH and insulin-like growth factor-Ⅰ(IGF-Ⅰ).GH was not inhibited by oral glucose tolerance test.PRL concentration was over 100 ng/mL.Triiodothyronine(T_(3))and thyroxine(T_(4))were also elevated,while TSH was not inhibited.All pituitary adenomas in four cases were macroadenomas or giant adenomas,all of which were invasive growth,and one case developed pituitary stroke.Except for one patient who did not receive treatment in our hospital due to medical expenses,the remaining three patients underwent a combined treatment of medication and transnasal transsphenoidal pituitary adenoma resection.Among them,one patient had relief of central hyperthyroidism and hyperprolactinemia,but GH/IGF-Ⅰdid not meet the remission criteria.The other two patients had persistent non-resolution of at least 2 hormone axes.Conclusions Patients with GH/PRL/TSH mixed pituitary adenoma were mainly characterized by coarse facial features,GH/PRL/TSH hyperfunction,large adenoma volume,low biochemical remission after surgery combined with drug treatment,and poor clinical prognosis.