Comparison of clinical, hormonal, pathological and treatment outcomes of ectopic Cushing’s syndrome by sex: results of a multicenter study

IF 3.7 3区医学 Q2 Medicine

Endocrine Pub Date : 2024-09-17 DOI:10.1007/s12020-024-04004-x

Sema Ciftci, Nusret Yilmaz, Ozlem Soyluk Selcukbiricik, Zeliha Hekimsoy, Asena Gökcay Canpolat, Senay Topsakal, Guzin Fidan Yaylali, Fuat Misiroglu, Nurdan Gul, Ayse Kubat Uzum, Ayşa Hacioglu, Goknur Yorulmaz, Hatice Ozisik, Banu Sarer Yurekli, Zafer Pekkolay, Seda Turgut, Zuleyha Karaca, Cevdet Duran, Medine Nur Kebapci, Sema Yarman

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引用次数: 0

Abstract

Objective

To compare clinical and hormonal data, neuroendocrine neoplasia (NEN) localization, treatment, and survival outcomes in ectopic Cushing’s syndrome (ECS) by sex.

Methods

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.

Results

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.

Conclusion

While ECS has a similar disease course in many aspects in males and females, hyperglycemia and osteoporosis are more severe in males.

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异位库欣综合征的临床、激素、病理和治疗效果的性别比较：一项多中心研究的结果

摘要] 目的比较不同性别异位库欣综合征（ECS）患者的临床和激素数据、神经内分泌肿瘤（NEN）定位、治疗和生存结果。对患者的临床和激素特征、肿瘤成像、病理结果、治疗方式和病程进行了评估。结果 28 名女性和 26 名男性 ECS 患者的诊断平均年龄、临床特征和激素评估结果相似。然而，胰岛素依赖型糖尿病（p = 0.04）和伴有骨折的骨质疏松症在男性中更为常见（p = 0.03）。发现女性中DHEA-S水平升高超过正常值上限的患者较多，而男性中枢性甲状腺功能减退症患者较多（p = 0.02）。在确诊时，36 例 NEN（68% 的女性和 69% 的男性）是局部性的。男性患小细胞肺癌的比例较高（P = 0.02），其他非小细胞肺癌的发生率没有差异。61%的女性和46%的男性接受了根治性手术。男女患者的肿瘤大小、Ki-67标记指数、ACTH免疫染色阳性率、局部淋巴结转移率和远处转移率相似。在随访中，经过药物治疗和/或双侧肾上腺切除术后，10名女性中有7人、8名男性中有4人的肿瘤变得明显。结论虽然ECS在许多方面与男性和女性的病程相似，但男性的高血糖和骨质疏松症更为严重。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Endocrine 医学-内分泌学与代谢

CiteScore

6.40

自引率

5.40%

发文量

期刊介绍： Well-established as a major journal in today’s rapidly advancing experimental and clinical research areas, Endocrine publishes original articles devoted to basic (including molecular, cellular and physiological studies), translational and clinical research in all the different fields of endocrinology and metabolism. Articles will be accepted based on peer-reviews, priority, and editorial decision. Invited reviews, mini-reviews and viewpoints on relevant pathophysiological and clinical topics, as well as Editorials on articles appearing in the Journal, are published. Unsolicited Editorials will be evaluated by the editorial team. Outcomes of scientific meetings, as well as guidelines and position statements, may be submitted. The Journal also considers special feature articles in the field of endocrine genetics and epigenetics, as well as articles devoted to novel methods and techniques in endocrinology. Endocrine covers controversial, clinical endocrine issues. Meta-analyses on endocrine and metabolic topics are also accepted. Descriptions of single clinical cases and/or small patients studies are not published unless of exceptional interest. However, reports of novel imaging studies and endocrine side effects in single patients may be considered. Research letters and letters to the editor related or unrelated to recently published articles can be submitted. Endocrine covers leading topics in endocrinology such as neuroendocrinology, pituitary and hypothalamic peptides, thyroid physiological and clinical aspects, bone and mineral metabolism and osteoporosis, obesity, lipid and energy metabolism and food intake control, insulin, Type 1 and Type 2 diabetes, hormones of male and female reproduction, adrenal diseases pediatric and geriatric endocrinology, endocrine hypertension and endocrine oncology.