Frontiers in Endocrinology (Mar 2023)

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

  • Arnika Wydra,
  • Karolina Cylke-Falkowska,
  • Izabella Czajka-Oraniec,
  • Agnieszka Kolasińska-Ćwikła,
  • Agnieszka Kolasińska-Ćwikła,
  • Jarosław Ćwikła,
  • Jarosław Ćwikła,
  • Wojciech Zgliczyński,
  • Maria Stelmachowska-Banaś

DOI
https://doi.org/10.3389/fendo.2023.1135016
Journal volume & issue
Vol. 14

Abstract

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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.

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