Vyberg M, Sestoft L
Am J Clin Pathol. 1986 Oct;86(4):541-5. doi: 10.1093/ajcp/86.4.541.
A case of Cushing's syndrome in a 31-year-old woman is presented. The resected left adrenal gland revealed a tumor consisting of cortical cells intermingled with myelolipomatous tissue comparable to that of normal bone marrow. The adjacent cortex was atrophic. Postoperative plasma cortisol concentrations have remained quite low. Previously, 28 cases of surgically removed adrenocortical tumors with a main diagnosis of myelolipoma have been published. Of these, three cases (two pituitary Cushing's disease, one steroid 21-hydroxylase deficiency) were associated with endocrine dysfunction. The combination of a myelolipoma and a true adenoma has only been described once before (in a case of virilization) and never in connection with Cushing's syndrome. The etiology of myelolipoma is discussed, and a local trigger mechanism related to adrenocortical growth disturbances is suggested.
本文报告了一名31岁女性库欣综合征病例。切除的左侧肾上腺显示一个肿瘤,由与正常骨髓类似的皮质细胞和髓质脂肪瘤组织混合组成。相邻皮质萎缩。术后血浆皮质醇浓度一直很低。此前,已发表28例以髓质脂肪瘤为主要诊断的手术切除肾上腺皮质肿瘤病例。其中3例(2例垂体库欣病,1例类固醇21-羟化酶缺乏症)伴有内分泌功能障碍。髓质脂肪瘤与真正腺瘤的组合此前仅被描述过一次(在一例男性化病例中),从未与库欣综合征相关联。本文讨论了髓质脂肪瘤的病因,并提出了一种与肾上腺皮质生长紊乱相关的局部触发机制。