异位库欣氏综合征严重发作于转变性别的男性，伴有转移性胃泌素瘤和肾上腺肿瘤——一份病例报告和文献综述。
Severe ectopic Cushing syndrome in a transgender man with a metastatic gastrinoma and an adrenal tumor-A case report and review of the literature.
发表日期：2023
作者：
Arnika Wydra, Karolina Cylke-Falkowska, Izabella Czajka-Oraniec, Agnieszka Kolasińska-Ćwikła, Jarosław Ćwikła, Wojciech Zgliczyński, Maria Stelmachowska-Banaś
来源：
Frontiers in Endocrinology
摘要：
一位38岁的跨性别男性因ACTH依赖性高皮质醇血症严重而入院内分泌科。怀疑是由PanNEN引起的ACTH异位分泌。术前经过美曲酮治疗后，该患者符合双侧肾上腺切除的条件。最终，该患者仅摘除左侧肾上腺，结果ACTH和皮质醇水平显著降低，导致病情改善。病理报告显示肾上腺皮质腺瘤，ACTH染色呈阳性。同时检查肝脏病变活检结果证实了转移性NEN G2和ACTH免疫染色呈阳性。我们探讨了性别肯定激素治疗与疾病发生及其快速进展之间的相关性。这可能是描述跨性别患者中胃泌素瘤和异位库欣病共存的首例。Copyright © 2023 Wydra, Cylke-Falkowska, Czajka-Oraniec, Kolasińska-Ćwikła, Ćwikła, Zgliczyński and Stelmachowska-Banaś.
A 38-year-old transgender man with advanced metastatic functional pancreatic neuroendocrine neoplasm (PanNEN) gastrinoma was admitted to the Department of Endocrinology due to severe ACTH-dependent hypercortisolemia. An ectopic production of ACTH by PanNEN was suspected. The patient qualified for bilateral adrenalectomy after preoperative treatment with metyrapone. Finally, the patient underwent resection of the left adrenal gland with the tumor only, which surprisingly resulted in a significant decrease in ACTH and cortisol levels, leading to clinical improvement. Pathology report revealed an adenoma of the adrenal cortex with positive ACTH staining. The result of the simultaneous liver lesion biopsy confirmed a metastatic NEN G2 with positive ACTH immunostaining as well. We looked for a correlation between gender-affirming hormone treatment and the onset of the disease and its rapid progression. This may be the first case describing the coexistence of gastrinoma and ectopic Cushing disease in a transsexual patient.Copyright © 2023 Wydra, Cylke-Falkowska, Czajka-Oraniec, Kolasińska-Ćwikła, Ćwikła, Zgliczyński and Stelmachowska-Banaś.