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Peer-reviewedCC BY-NCCaspian journal of internal medicine

First case report of ectopic Cushing's syndrome secondary to synchronous mediastinal neuroendocrine tumors.

  • Edita Guadalupe Ruiz-Huaranga1,2
  • Maria Del Pilar Quinones Avila3
  • Rosa Laurie Marcilla Truyenque1
  • Marcio Jose Concepcion-Zavaleta4
  • Jose Paz-Ibarra1,5
  • Jenyfer Fuentes-Mendoza4
  • Juan Eduardo Quiroz-Aldave6
6 affiliations
  1. 1Division of Endocrinology, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
  2. 2Universidad Peruana Cayetano Heredia, Lima, Peru.
  3. 3Division of Anatomic Pathology, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
  4. 4Grupo de Investigacion en Neurociencias, Metabolismo, Efectividad Clinicay Salud Publica, Universidad Científica del Sur, Lima, Peru.
  5. 5School of Medicine, Universidad Nacional Mayor de San Marcos, Lima, Peru.
  6. 6Division of Research, Hospital de Apoyo Chepen, Chepen, Peru.
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Abstract

Background

Ectopic Cushing's syndrome (ECS) is a rare cause of hypercortisolism resulting from non-pituitary adrenocorticotropic hormone (ACTH) secretion, most associated with neuroendocrine tumors (NETs) of pulmonary or gastrointestinal origin. Mediastinal NETs are an extremely rare source.Case presentation

We report the case of a 23-year-old male with no relevant medical history, presenting with progressive muscle weakness, hyperpigmentation, weight gain, and signs of severe hypercortisolism, including moon facies, ecchymosis, hypertension, hyperglycemia, and hypokalemia. Hormonal tests confirmed ECS with markedly elevated ACTH and cortisol levels. Imaging revealed two anterior mediastinal masses. Histopathological analysis of the surgical specimens confirmed ACTH-producing atypical NETs.Conclusions

This case highlights the importance of considering ectopic ACTH secretion from mediastinal neuroendocrine tumors in the differential diagnosis of severe, rapidly progressive hypercortisolism. Early recognition and timely surgical management can prevent life-threatening complications and improve patient outcomes, even in rare and aggressive presentations.

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Cite

BibTeXRIS
BibTeX
@article{ruizhuaranga2026first,
  title = {{First case report of ectopic Cushing's syndrome secondary to synchronous mediastinal neuroendocrine tumors.}},
  author = {Edita Guadalupe Ruiz-Huaranga and Maria Del Pilar Quinones Avila and Rosa Laurie Marcilla Truyenque and Marcio Jose Concepcion-Zavaleta and Jose Paz-Ibarra and Jenyfer Fuentes-Mendoza and Juan Eduardo Quiroz-Aldave},
  journal = {Caspian journal of internal medicine},
  year = {2026},
  month = sep,
  doi = {10.22088/cjim.17.3.681},
  url = {https://europepmc.org/article/MED/42924379},
  note = {CC BY-NC},
}
RIS
TY  - JOUR
TI  - First case report of ectopic Cushing's syndrome secondary to synchronous mediastinal neuroendocrine tumors.
AU  - Edita Guadalupe Ruiz-Huaranga
AU  - Maria Del Pilar Quinones Avila
AU  - Rosa Laurie Marcilla Truyenque
AU  - Marcio Jose Concepcion-Zavaleta
AU  - Jose Paz-Ibarra
AU  - Jenyfer Fuentes-Mendoza
AU  - Juan Eduardo Quiroz-Aldave
PY  - 2026
DA  - 2026/09/01
JO  - Caspian journal of internal medicine
DO  - 10.22088/cjim.17.3.681
UR  - https://europepmc.org/article/MED/42924379
AB  - Background  Ectopic Cushing's syndrome (ECS) is a rare cause of hypercortisolism resulting from non-pituitary adrenocorticotropic hormone (ACTH) secretion, most associated with neuroendocrine tumors (NETs) of pulmonary or gastrointestinal origin. Mediastinal NETs are an extremely rare source.Case presentation  We report the case of a 23-year-old male with no relevant medical history, presenting with progressive muscle weakness, hyperpigmentation, weight gain, and signs of severe hypercortisolism, including moon facies, ecchymosis, hypertension, hyperglycemia, and hypokalemia. Hormonal tests confirmed ECS with markedly elevated ACTH and cortisol levels. Imaging revealed two anterior mediastinal masses. Histopathological analysis of the surgical specimens confirmed ACTH-producing atypical NETs.Conclusions  This case highlights the importance of considering ectopic ACTH secretion from mediastinal neuroendocrine tumors in the differential diagnosis of severe, rapidly progressive hypercortisolism. Early recognition and timely surgical management can prevent life-threatening complications and improve patient outcomes, even in rare and aggressive presentations.
SN  - 2008-6164
SN  - 2008-6172
ER  -