Bakirkoy Dr. Sadi Konuk Training and Research Hospital, Department of Endocrinology and Metabolism, University of Health Sciences, Istanbul, Turkey.
School of Medicine, Department of Endocrinology and Metabolism, Akdeniz University, Antalya, Turkey.
Endocrine. 2024 Dec;86(3):1148-1155. doi: 10.1007/s12020-024-04004-x. Epub 2024 Sep 17.
To compare clinical and hormonal data, neuroendocrine neoplasia (NEN) localization, treatment, and survival outcomes in ectopic Cushing's syndrome (ECS) by sex.
Eleven experienced centers from our country participated in this retrospective study. The clinical and hormonal features, tumor imaging, pathological results, treatment modalities, and disease courses of the patients were evaluated.
28 female and 26 male patients with ECS were compared. The mean age at diagnosis, clinical characteristics, and hormonal evaluation results were similar. However, insulin-requiring diabetes mellitus (p = 0.04) and osteoporosis with fractures were more common in males (p = 0.03). While more patients with increased DHEA-S levels than the upper limit of normal were found to be higher in females, central hypothyroidism were higher in males (p = 0.02). At the diagnosis, 36 NENs (68% of females and 69% of males) were localized. Small cell lung carcinoma was higher in males (p = 0.02), and the frequency of other NENs was not different. Curative surgery was performed on 61% of females and 46% of males. Tumor size, Ki-67 labeling index, positive ACTH immunostaining, local lymph node and distant metastasis rates were similar in both sexes. In the follow-up, the tumor became visible in 7 of 10 females and 4 of 8 males after medical treatment and/or bilateral adrenalectomy. The remission rates (65% of females, 62% of males) and NEN-related death rates (14% of females, 30% of males) were similar.
While ECS has a similar disease course in many aspects in males and females, hyperglycemia and osteoporosis are more severe in males.
比较性别对异位库欣综合征（ECS）的临床和激素数据、神经内分泌肿瘤（NEN）定位、治疗和生存结局的影响。
我国 11 个有经验的中心参与了这项回顾性研究。评估了患者的临床和激素特征、肿瘤影像学、病理结果、治疗方式和疾病过程。
比较了 28 例女性和 26 例男性 ECS 患者。诊断时的平均年龄、临床特征和激素评估结果相似。然而，男性更常见需要胰岛素的糖尿病（p=0.04）和骨质疏松伴骨折（p=0.03）。虽然女性中发现更多 DHEA-S 水平升高超过正常值上限的患者，但男性中中枢性甲状腺功能减退症更高（p=0.02）。在诊断时，36 例 NEN（68%的女性和 69%的男性）定位。男性中更常见小细胞肺癌（p=0.02），其他 NEN 的发生率无差异。对 61%的女性和 46%的男性进行了治愈性手术。肿瘤大小、Ki-67 标记指数、促肾上腺皮质激素免疫染色阳性、局部淋巴结和远处转移率在两性之间相似。在随访中，10 例女性中有 7 例和 8 例男性中有 4 例在药物治疗和/或双侧肾上腺切除后肿瘤可见。缓解率（女性 65%，男性 62%）和 NEN 相关死亡率（女性 14%，男性 30%）相似。
尽管 ECS 在许多方面在男性和女性中具有相似的病程，但男性的高血糖和骨质疏松更为严重。
