Babaya Naru, Noso Shinsuke, Hiromine Yoshihisa, Taketomo Yasunori, Niwano Fumimaru, Monobe Keisuke, Imamura Shuzo, Ueda Kazuki, Yamazaki Yuto, Sasano Hironobu, Ikegami Hiroshi
Department of Endocrinology, Metabolism and Diabetes, Kindai University Faculty of Medicine, Osaka-sayama, Osaka 589-8511, Japan.
Department of Surgery, Kindai University Faculty of Medicine, Osaka-sayama, Osaka 589-8511, Japan.
J Endocr Soc. 2021 Aug 23;5(11):bvab143. doi: 10.1210/jendso/bvab143. eCollection 2021 Nov 1.
Adrenocortical carcinoma (ACC) is a rare tumor, and some histological variants (oncocytic, myxoid, and sarcomatoid ACCs) have been reported in addition to the conventional ACC. Among these subtypes, oncocytic ACC is histologically characterized by the presence of abundant eosinophilic granular cytoplasm in the carcinoma cells owing to the accumulation of mitochondria, which generally yields high F-fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET). Herein, we report the case of a 21-year-old woman with oncocytic ACC with low FDG uptake on PET scan. Her circulating levels of androgens were high, and androgen-synthesis enzymes were detected in carcinoma cells. The patient also had hypocholesterolemia. However, glucose transporter 1 (GLUT1) was not detected in the tumor, which was considered to account for the low FDG uptake by the tumor. To the best of our knowledge, this is the first case of low FDG uptake by oncocytic ACC without GLUT1 expression. Additionally, since hypocholesterolemia was reported in 3 previous reports of androgen-producing tumors, a possible correlation between androgenicity in adrenal tumors and the development of hypocholesterolemia could be postulated; however, further investigations are needed for clarification. This case highlights important information regarding the diversity of ACC and its impact on hypocholesterolemia.
肾上腺皮质癌（ACC）是一种罕见肿瘤，除了传统的ACC外，还报道了一些组织学变异型（嗜酸性细胞型、黏液样型和肉瘤样型ACC）。在这些亚型中，嗜酸性细胞型ACC在组织学上的特征是癌细胞中存在丰富的嗜酸性颗粒状细胞质，这是由于线粒体的积累所致，这通常会导致正电子发射断层扫描（PET）上氟脱氧葡萄糖（FDG）摄取量高。在此，我们报告一例21岁患有嗜酸性细胞型ACC的女性病例，其PET扫描显示FDG摄取量低。她的循环雄激素水平很高，并且在癌细胞中检测到了雄激素合成酶。该患者还患有低胆固醇血症。然而，在肿瘤中未检测到葡萄糖转运蛋白1（GLUT1），这被认为是肿瘤FDG摄取量低的原因。据我们所知，这是首例无GLUT1表达的嗜酸性细胞型ACC出现低FDG摄取的病例。此外，由于在之前3篇关于产生雄激素肿瘤的报道中均有低胆固醇血症的记载，因此可以推测肾上腺肿瘤的雄激素性与低胆固醇血症的发生之间可能存在关联；然而，需要进一步研究以阐明。该病例突出了关于ACC多样性及其对低胆固醇血症影响的重要信息。
