Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndrome
Ectopic adrenocorticotropic hormone secretion (EAS) is responsible for approximately 10%–18% of Cushing’s syndrome cases. Thymic neuroendocrine tumors (NETs) comprise 5%–16% of EAS; therefore, they are very rare and the data about this particular tumors is scarce. We present a case of a 34-year-old...
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Frontiers Media S.A.
2025-02-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2025.1492187/full |
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| author | Aleksandra Zdrojowy-Wełna Marek Bolanowski Joanna Syrycka Joanna Syrycka Aleksandra Jawiarczyk-Przybyłowska Justyna Kuliczkowska-Płaksej |
| author_facet | Aleksandra Zdrojowy-Wełna Marek Bolanowski Joanna Syrycka Joanna Syrycka Aleksandra Jawiarczyk-Przybyłowska Justyna Kuliczkowska-Płaksej |
| author_sort | Aleksandra Zdrojowy-Wełna |
| collection | DOAJ |
| description | Ectopic adrenocorticotropic hormone secretion (EAS) is responsible for approximately 10%–18% of Cushing’s syndrome cases. Thymic neuroendocrine tumors (NETs) comprise 5%–16% of EAS; therefore, they are very rare and the data about this particular tumors is scarce. We present a case of a 34-year-old woman with a rapid onset of severe hypercortisolism in April 2016. After initial treatment with a steroid inhibitor (ketoconazole) and diagnostics including 68Ga DOTA-TATE PET/CT, it was shown to be caused by a small thymic NET. After a successful surgery and the resolution of all symptoms, there was a recurrence after 5 years of observation caused by a metastasis to the breast, shown in the 68Ga DOTA-TATE PET/CT result and confirmed with a breast biopsy. Treatment with a steroid inhibitor (metyrapone) and tumor resection were again curative. The last disease relapse appeared 7 years after the initial treatment, with severe hypercortisolism treated with osilodrostat. There was a local recurrence in the mediastinum, and a thoracoscopic surgery was performed with good clinical and biochemical effect. The patient remains under careful follow-up. Our case stays in accordance with recent literature data, showing that patients with thymic NETs are younger than previously considered and that the severity of hypercortisolism does not correlate with the tumor size. The symptoms of EAS associated with thymic NET may develop rapidly and may be severe as in our case. Nuclear medicine improves the effectiveness of the tumor search, which is crucial in successful EAS therapy. Our case also underlines the need for lifelong monitoring of patients with thymic NETs and EAS. |
| format | Article |
| id | doaj-art-02726b9457ca45feb803f8d7f1fd898a |
| institution | OA Journals |
| issn | 2234-943X |
| language | English |
| publishDate | 2025-02-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj-art-02726b9457ca45feb803f8d7f1fd898a2025-08-20T02:01:20ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2025-02-011510.3389/fonc.2025.14921871492187Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndromeAleksandra Zdrojowy-Wełna0Marek Bolanowski1Joanna Syrycka2Joanna Syrycka3Aleksandra Jawiarczyk-Przybyłowska4Justyna Kuliczkowska-Płaksej5Department and Clinic of Endocrinology and Internal Medicine, Wroclaw Medical University, Wroclaw, PolandDepartment and Clinic of Endocrinology and Internal Medicine, Wroclaw Medical University, Wroclaw, PolandDepartment and Clinic of Endocrinology and Internal Medicine, Wroclaw Medical University, Wroclaw, PolandAffidea PET/CT Diagnostic Center, Wrocław, PolandDepartment and Clinic of Endocrinology and Internal Medicine, Wroclaw Medical University, Wroclaw, PolandDepartment and Clinic of Endocrinology and Internal Medicine, Wroclaw Medical University, Wroclaw, PolandEctopic adrenocorticotropic hormone secretion (EAS) is responsible for approximately 10%–18% of Cushing’s syndrome cases. Thymic neuroendocrine tumors (NETs) comprise 5%–16% of EAS; therefore, they are very rare and the data about this particular tumors is scarce. We present a case of a 34-year-old woman with a rapid onset of severe hypercortisolism in April 2016. After initial treatment with a steroid inhibitor (ketoconazole) and diagnostics including 68Ga DOTA-TATE PET/CT, it was shown to be caused by a small thymic NET. After a successful surgery and the resolution of all symptoms, there was a recurrence after 5 years of observation caused by a metastasis to the breast, shown in the 68Ga DOTA-TATE PET/CT result and confirmed with a breast biopsy. Treatment with a steroid inhibitor (metyrapone) and tumor resection were again curative. The last disease relapse appeared 7 years after the initial treatment, with severe hypercortisolism treated with osilodrostat. There was a local recurrence in the mediastinum, and a thoracoscopic surgery was performed with good clinical and biochemical effect. The patient remains under careful follow-up. Our case stays in accordance with recent literature data, showing that patients with thymic NETs are younger than previously considered and that the severity of hypercortisolism does not correlate with the tumor size. The symptoms of EAS associated with thymic NET may develop rapidly and may be severe as in our case. Nuclear medicine improves the effectiveness of the tumor search, which is crucial in successful EAS therapy. Our case also underlines the need for lifelong monitoring of patients with thymic NETs and EAS.https://www.frontiersin.org/articles/10.3389/fonc.2025.1492187/fullectopic Cushing`s syndromethymic neuroendocrine tumorthymic NETectopic ACTH secretioncase report |
| spellingShingle | Aleksandra Zdrojowy-Wełna Marek Bolanowski Joanna Syrycka Joanna Syrycka Aleksandra Jawiarczyk-Przybyłowska Justyna Kuliczkowska-Płaksej Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndrome Frontiers in Oncology ectopic Cushing`s syndrome thymic neuroendocrine tumor thymic NET ectopic ACTH secretion case report |
| title | Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndrome |
| title_full | Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndrome |
| title_fullStr | Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndrome |
| title_full_unstemmed | Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndrome |
| title_short | Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing’s syndrome |
| title_sort | case report thymic neuroendocrine tumor with metastasis to the breast causing ectopic cushing s syndrome |
| topic | ectopic Cushing`s syndrome thymic neuroendocrine tumor thymic NET ectopic ACTH secretion case report |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2025.1492187/full |
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