儿童和青春期垂体促肾上腺皮质激素微腺瘤的诊断和治疗

Diagnosis and treatment of pediatric and hebetic adrenocorticotropic hormone secreting pituitary microadenoma

目的探讨儿童和青春期垂体促肾上腺皮质激素(ACTH)微腺瘤的诊断和治疗方法。方法回顾性分析10例儿童和青春期垂体ACTH微腺瘤患者的病例资料,根据临床症状、生物化学检查和影像学检查结果进行诊断,其中5例行双侧岩下静脉窦采血(BIPSS)。所有患者均在显微镜下行经蝶垂体腺瘤切除术;术后肿瘤标本行免疫组织化学检查。结果术后免疫组织化学检查示ACTH阳性8例,阴性2例。随访12～63个月,治愈7例(70%),缓解1例(10%),复发2例(20%)。结论BIPSS有助于儿童和青春期垂体ACTH微腺瘤的诊断和肿瘤定位;经蝶垂体腺瘤切除术是儿童和青春期垂体ACTH微腺瘤的首选治疗方法。

Objective To investigate the methods of diagnosis and treatment of pediatric and hebetic adrenocorticotropic hormone （ACTH） secreting pituitary microadenoma. Methods The data of 10 patients with ACTH secreting pituitary microadenoma were retrospectively analysed. ACTH secreting pituitary microadenoma was diagnosed by clinical manifes- tations, biochemical and imaging findings, und 5 patients underwent bilateral inferior petrosal sinus sampling （BIPSS）. Transsphenoidal surgery was performed on all the patients under microscope, and the tumor specimens were detected by immunohistochemistry. Results Immunohistochemical examination revealed that ACTH was positive in 8 cases and negative in 2 cases. Patients were followed up for 12 to 63 months, 7 cases （70%） were cured, one （10%） achieved remission, and the other two （20%） experienced recurrence. Conclusion BIPSS is helpful in the diagnosis and localization of pediatric and hebetic ACTH pituitary microadenoma, and transsphenoidal surgery is the optimal choice of treatment.