Lonergan Eibhlín Marie, Tan Lok Yi Joyce, O'Sullivan Adrian, Kanazaki Keizo, Morita Miwa, O'Halloran Domhnall
Department of Endocrinology and Diabetes, Cork University Hospital, Cork T12 DFK4, Ireland.
Department of Surgery, Cork University Hospital, Cork T12 DFK4, Ireland.
JCEM Case Rep. 2025 Apr 23;3(6):luaf078. doi: 10.1210/jcemcr/luaf078. eCollection 2025 Jun.
Non-islet cell tumor hypoglycemia as a result of insulin-like growth factor (IGF)-2 secretion is rare. A 59-year-old woman was referred with postmenopausal bleeding due to endometrial hyperplasia. Serum testosterone, estradiol, and adrenal androgens were elevated with suppressed gonadotropin concentrations. Cross-sectional imaging demonstrated a large left adrenal mass. The patient subsequently presented acutely with hypoglycemia. During a supervised fast, symptomatic hypoglycemia occurred within 5 hours. Serum samples drawn prior to hypoglycemia correction revealed an elevated IGF-2:IGF-1 ratio of 60.7 (normal <10) with low paired C-peptide and insulin, consistent with an IGF-2-secreting tumor. Hypoglycemia was managed with low-glycemic index foods and radical surgical excision was undertaken. Postoperative pathology revealed an adrenocortical carcinoma (ACC); Ki67 12%; IGF-2 positive immunostaining. This case demonstrates a rare IGF-2-secreting ACC causing clinically significant hypoglycemia with positive immunostaining for IGF-2 in addition to biochemical hyperandrogenism resulting in endometrial hyperplasia and postmenopausal bleeding. This case encompasses 2 unique syndromes from the cosecretion of both peptide factor and steroid hormone.
因胰岛素样生长因子（IGF）-2分泌导致的非胰岛细胞瘤低血糖症较为罕见。一名59岁女性因子宫内膜增生出现绝经后出血前来就诊。血清睾酮、雌二醇以及肾上腺雄激素水平升高，促性腺激素浓度降低。横断面成像显示左侧肾上腺有一个大肿块。该患者随后急性发作低血糖。在严密监测的禁食期间，5小时内出现了有症状的低血糖。在低血糖纠正前采集的血清样本显示，IGF-2与IGF-1的比值升高至60.7（正常<10），同时配对的C肽和胰岛素水平较低，这与分泌IGF-2的肿瘤相符。通过低血糖指数食物对低血糖进行了处理，并进行了根治性手术切除。术后病理显示为肾上腺皮质癌（ACC）；Ki67为12%；IGF-2免疫染色呈阳性。该病例展示了一种罕见的分泌IGF-2的ACC，除了导致生化性高雄激素血症进而引起子宫内膜增生和绝经后出血外，还导致具有临床意义的低血糖，且IGF-2免疫染色呈阳性。该病例涵盖了由肽因子和类固醇激素共同分泌引起的两种独特综合征。
