Rockall Andrea G, Babar Syed A, Sohaib S A Aslam, Isidori Andrea M, Diaz-Cano Salvador, Monson John P, Grossman Ashley B, Reznek Rodney H
Department of Academic Radiology, St Bartholomew's Hospital, Dominion House, St Bartholomew's Close, London EC1A 7ED, England.
Radiographics. 2004 Mar-Apr;24(2):435-52. doi: 10.1148/rg.242035092.
Adrenocorticotropic hormone (ACTH)-independent hypercortisolism accounts for 15%-20% of cases of Cushing syndrome and always arises from primary adrenal disease. Computed tomographic (CT) and magnetic resonance (MR) imaging findings in 37 patients with primary adrenal Cushing syndrome were analyzed and correlated with pathologic findings. Hyperfunctioning adenomas (n = 24), together with functioning carcinomas (n = 10), accounted for 92% of cases. Adenomas had a significantly smaller mean size (3.5 vs 14.5 cm) and lower mean unenhanced CT attenuation value (11 vs 28 HU) than did carcinomas. The presence of necrosis, hemorrhage, and calcification favored a diagnosis of carcinoma. Six of 10 carcinoma patients had metastases at presentation. Two adenomas were seen within a myelolipoma, which was recognized at both CT and MR imaging due to its fat content, and two adenomas were of uncertain malignant potential. Bilateral disease--primary pigmented nodular adrenal dysplasia (PPNAD) (n = 2) and ACTH-independent macronodular adrenal hyperplasia (AIMAH) (n = 1)--had characteristic imaging features. In PPNAD, multiple tiny (2-5-mm) nodules were visible bilaterally, with no overall glandular enlargement and normal intervening adrenal tissue. In AIMAH, both glands were grossly enlarged and contained nodules up to 3 cm in diameter. Familiarity with the range of imaging appearances of the adrenal glands in primary adrenal Cushing syndrome may help establish the underlying diagnosis.
促肾上腺皮质激素（ACTH）非依赖性皮质醇增多症占库欣综合征病例的15% - 20%，且总是由原发性肾上腺疾病引起。对37例原发性肾上腺库欣综合征患者的计算机断层扫描（CT）和磁共振（MR）成像结果进行了分析，并与病理结果进行了对比。高功能腺瘤（n = 24）和功能性癌（n = 10）占病例的92%。腺瘤的平均大小（3.5 cm对14.5 cm）和平均CT平扫衰减值（11 HU对28 HU）均显著小于癌。坏死、出血和钙化的存在有利于癌的诊断。10例癌患者中有6例在就诊时已有转移。在髓样脂肪瘤内可见2个腺瘤，因其脂肪成分在CT和MR成像上均能被识别，另外2个腺瘤的恶性潜能不确定。双侧病变——原发性色素沉着性结节性肾上腺发育不良（PPNAD）（n = 2）和ACTH非依赖性大结节性肾上腺增生（AIMAH）（n = 1）——具有特征性的影像学表现。在PPNAD中，双侧可见多个微小（2 - 5 mm）结节，肾上腺无整体增大，中间肾上腺组织正常。在AIMAH中，双侧肾上腺均明显增大，含有直径达3 cm的结节。熟悉原发性肾上腺库欣综合征中肾上腺的一系列影像学表现可能有助于确立潜在的诊断。
