经蝶手术治疗临床表现为库欣病的垂体ACTH腺瘤(附29例报道)

The Diagnosis and Surgical Treatment of Pituitary ACTH- secreting Cushing’s Adenoma

目的：分析临床表现为库欣病的垂体促肾上腺皮质激素（ACTH）腺瘤的诊断和手术治疗疗效,探讨影响疗效的因素。方法：回顾分析29例库欣病患者的临床资料。术前MRI显示垂体大腺瘤2例、微腺瘤14例、可疑微腺瘤11例、未见肿瘤征象者2例。其中14例行双侧岩下窦采血（BIPSS）检查。可疑微腺瘤和未见肿瘤征象13例术前均依据地塞米松抑制试验和BIPSS诊断为库欣病。29例患者均行经右侧单鼻孔-蝶窦手术切除肿瘤。以术后2 d内上午8：00血皮质醇低于5μg·dL-1（1μg·dL-1=27.6 nmol.L-1）、24 h尿游离皮质醇正常、原有症状缓解或消失作为术后即刻生化缓解（IPBR）标准。结果：29例患者均病理证实为垂体ACTH腺瘤。23/29例（79.3%）症状缓解,术后皮质醇水平介于0.47~4.35μg·dL-1,平均为2.37μg·dL-1。术前MRI显示明确腺瘤患者的缓解率[15/16例（93.8%）]高于可疑微腺瘤和未见肿瘤患者的缓解率[8/13例（61.5%,P〈0.05）];术前MRI提示可疑微腺瘤和未见肿瘤的13例患者中,行BIPSS检查患者的缓解率[7/9例（77.8%）]高于未行BIPSS检查患者[1/4例（25.0%）]。14例行BIPSS检查者中,9例术中所见病灶位置与术前BIPSS提示位置相符9/14例（64.3%）。结论：BIPSS有助于库欣病术前ACTH微腺瘤的鉴别诊断和肿瘤定位;经蝶入路是治疗垂体ACTH微腺瘤手术的首选方法,术后未缓解或复发病例可考虑再次手术,术前MRI显示肿瘤的患者提示预后良好。

Aim： To demonstrate the results following surgical treatment of pituitary ACTH-secreting Cushing’s adenoma,analyze the diagnostic modalities of this disease and assess the the factors that influence the outcome.Methods： The clinical data of 29 patients with pituitary ACTH-secreting adenoma were analyzed retrospectively.The preoperative MRI showed that 2 were macroadenoma,14 microadenoma,11 unconfirmed microadenoma and 2 unvisible adenomas.14 patients underwent bilateral inferior petrosal sinus sampling（BIPSS）.All the patients were operated via trans-sphenoidal approach.The immediate postoperative biochemical remission（IPBR） was defined as a lower-than 5 μg.dL-1morning serum cortisone level and normal 24 h-urinary free cortisone （24 h-UFC） within 2 days after operation.Results： All the patients were pathologically diagnosed as ACTHsecreting adenomas.The IPBR rate was 79.3%（23/29）,the postoperative free cortisone level of the patients ranged between 0.47~4.35 μg.dL-1（mean 2.37 μg.dL-1）.The patients with positive MRI results achieved higher IPBR rate than those with negative MRI results（93.75% vs 61.5%）.In patients with negative MRI results,the patients underwent（BIPSS） achieved higher IPBR rate than those did not undergo BIPSS（77.8% vs 25%）.9 patients in 14（9/14,64.3%） were correctly localized by BIPSS.Conclusion： BIPSS helps localize the lesion preoperatively and possibly helps improve the treatment effect of pituitaryACTH-secreting adenoma.Trans-sphenoidal operation is a preferred treatment of pituitary ACTH-secreting adenoma.Reoperation can be considered for recurrent or refractory cases.Positive preoperative MRI is a good predictive index of remission.