Aiba M
Department of Surgical Pathology, Daini Hospital, Tokyo Women's Medical College.
Nihon Naibunpi Gakkai Zasshi. 1994 Jan 20;70(1):37-42. doi: 10.1507/endocrine1927.70.1_37.
Six cases with ACTH-independent bilateral adrenocortical macronodular hyperplasia (AIMAH) were presented, and 21 reported cases were reviewed. Bilateral adrenal glands in AIMAH were greater than those in any other subtype of nodular hyperplasia. They were greater than 72 g in combined weight and were occupied by numerous yellow nodular/subnodular lesions. Histologically, hyperplastic lesions were composed of small compact, small clear, and usual clear cells. All of these three elements contained cells positive for PCNA but were negative for HLA-DR alpha. Small compact cells often showed positive staining for vimentin. Small cells showed underdeveloped organelles with rare nuclear grooving and were poor in lipochrome. The results are consistent with the view that AIMAH is primarily a nodular hyperplasia of the upper zona fasciculata and that it is a distinct subtype of Cushing's syndrome. Image analyses may play a major role on preoperative diagnosis of AIMAH. Bilateral adrenalectomy is the treatment of choice.
本文报告了6例促肾上腺皮质激素（ACTH）非依赖性双侧肾上腺皮质大结节增生（AIMAH）病例，并对21例已报道病例进行了回顾。AIMAH患者的双侧肾上腺大于其他任何类型的结节性增生患者。其双侧肾上腺总重量超过72g，内有大量黄色结节/小结节病变。组织学上，增生性病变由小致密细胞、小透明细胞和普通透明细胞组成。这三种细胞成分的细胞增殖核抗原（PCNA）均呈阳性，但人类白细胞抗原-DRα（HLA-DRα）均为阴性。小致密细胞波形蛋白常呈阳性染色。小细胞细胞器发育不全，核沟少见，脂褐素含量少。这些结果支持以下观点：AIMAH主要是肾上腺束状带上部的结节性增生，是库欣综合征的一种独特亚型。图像分析可能在AIMAH的术前诊断中起主要作用。双侧肾上腺切除术是首选的治疗方法。
