Shinojima H, Kakizaki H, Usuki T, Harabayashi T, Ameda K, Koyanagi T
Department of Urology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
J Urol. 2001 Nov;166(5):1639-42.
We report clinical findings in 5 patients with adrenocorticotropic hormone independent bilateral macronodular adrenocortical hyperplasia.
In 4 males and 1 female 32 to 61 years old (median age 50) we evaluated clinical symptoms, endocrinological and radiological characteristics, treatment modality and postoperative clinical course.
All cases presented with some features of Cushing's syndrome. Endocrinological examination revealed autonomous adrenal cortisol production with suppressed adrenocorticotropic hormone and a loss in the diurnal circadian rhythm of plasma cortisol. Abdominal computerized tomography showed bilateral enlargement of the adrenal glands with multiple nodules. 131Iodine labeled adosterol scintigraphy demonstrated remarkable bilateral uptake by the adrenal glands. The pituitary gland appeared normal on magnetic resonance imaging. Open unilateral complete adrenalectomy and contralateral partial adrenalectomy were performed in patient 1, open bilateral complete adrenalectomy was done in patients 2 and 3, and 2 and 1-stage laparoscopic bilateral complete adrenalectomy was performed in patients 4 and 5. Single removed adrenal glands weighed 32 to 108 gm. (median 60). The histological diagnosis was macronodular adrenocortical hyperplasia in all cases. Postoperative followup was 3 to 90 months. Clinical symptoms of Cushing's syndrome disappeared or improved after surgery in all cases.
Although adrenocorticotropic hormone independent bilateral macronodular adrenocortical hyperplasia is a rare form of Cushing's syndrome, physicians are advised to consider it when diagnosing and treating cases of Cushing's syndrome with enlarged bilateral adrenal glands. Bilateral complete adrenalectomy is currently recommended as the treatment of choice.
我们报告5例促肾上腺皮质激素非依赖性双侧大结节性肾上腺皮质增生患者的临床发现。
对4例男性和1例女性患者进行评估，年龄在32至61岁之间（中位年龄50岁），评估内容包括临床症状、内分泌和放射学特征、治疗方式及术后临床病程。
所有病例均表现出一些库欣综合征的特征。内分泌检查显示肾上腺皮质醇自主分泌，促肾上腺皮质激素受抑制，血浆皮质醇昼夜节律消失。腹部计算机断层扫描显示双侧肾上腺增大，有多个结节。131碘标记胆固醇闪烁扫描显示双侧肾上腺有明显摄取。磁共振成像显示垂体正常。患者1行开放性单侧肾上腺全切术及对侧肾上腺部分切除术，患者2和3行开放性双侧肾上腺全切术，患者4和5行2期及1期腹腔镜双侧肾上腺全切术。切除的单个肾上腺重量为32至108克（中位值60克）。所有病例的组织学诊断均为大结节性肾上腺皮质增生。术后随访3至90个月。所有病例术后库欣综合征的临床症状均消失或改善。
尽管促肾上腺皮质激素非依赖性双侧大结节性肾上腺皮质增生是库欣综合征的一种罕见形式，但建议医生在诊断和治疗双侧肾上腺增大的库欣综合征病例时考虑这种疾病。目前推荐双侧肾上腺全切术作为首选治疗方法。
