Severe ectopic Cushing syndrome in a transgender man with a metastatic gastrinoma and an adrenal tumor—A case report and review of the literature

Frontiers in Endocrinology

Endocrinology

Jarosław Ćwikła¹  Arnika Wydra²  Wojciech Zgliczyński²  Maria Stelmachowska-Banaś²  Izabella Czajka-Oraniec²  Agnieszka Kolasińska-Ćwikła³  Karolina Cylke-Falkowska⁴ 

[1] Department of Cardiology and Internal Medicine, Medical School University of Warmia and Mazury, Olsztyn, Poland;Diagnostic and Therapy Center – Gammed, Warsaw, Poland;Department of Endocrinology, Centre of Postgraduate Medical Education, Warsaw, Poland;Department of Oncology and Radiotherapy, Maria Skłodowska-Curie Memorial Cancer Center, Warsaw, Poland;Department of Radiology, Maria Skłodowska-Curie Memorial Cancer Center, Warsaw, Poland;I Department of Internal Medicine, Bielański Hospital, Warsaw, Poland;

关键词: pancreatic neuroendocrine neoplasm;    ectopic Cushing syndrome;    adrenal adenoma;    transgender;    gastrinoma;   

DOI  :  10.3389/fendo.2023.1135016

 received in 2022-12-31, accepted in 2023-02-27,  发布年份 2023

来源: Frontiers

【 摘 要 】

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.

【 授权许可】

Unknown   
Copyright © 2023 Wydra, Cylke-Falkowska, Czajka-Oraniec, Kolasińska-Ćwikła, Ćwikła, Zgliczyński and Stelmachowska-Banaś