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Typical Carcinoid Tumor Cases Causing Ectopic ACTH Syndrome: Dramatic Response to Surgery.

Aykut Kankoç1, Merve Şatır Türk1, Dilvin Özkan1

  • 1Department of Thoracic Surgery, Gazi University, Ankara, Turkey.

Portuguese Journal of Cardiac Thoracic and Vascular Surgery
|April 8, 2023
PubMed
Summary

Surgical removal of typical carcinoid tumors effectively treats ectopic ACTH syndrome (EAS). This approach normalizes adrenocorticotrophic hormone (ACTH) and cortisol levels, offering excellent long-term survival for Cushing

Keywords:
carcinoid tumorectopic ACTH syndromeectopic Cushing syndromesurgical resection

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Area of Science:

  • Endocrinology
  • Oncology
  • Thoracic Surgery

Background:

  • Cushing's Syndrome (CS) is typically caused by pituitary/adrenal adenomas.
  • A minority of CS cases involve ectopic Adrenocorticotrophic hormone (ACTH) secretion from extrapituitary neuroendocrine tumors (NET), particularly typical pulmonary/bronchial carcinoids, leading to ectopic ACTH syndrome (EAS).

Observation:

  • Two patients presented with symptoms of CS due to ectopic ACTH secretion.
  • Case 1: A 32-year-old female with CS symptoms and a left lower lobe lung lesion on CT.
  • Case 2: A 36-year-old male with a history of adrenalectomy for CS and adrenal insufficiency, presenting with a right lower lobe lung lesion on CT.

Findings:

  • Both patients underwent surgical resection of their respective lung tumors.
  • Post-surgery, both patients experienced regression of CS symptoms, and their ACTH and cortisol levels normalized.
  • Pathological examination confirmed the resected tumors as typical carcinoids.

Implications:

  • Surgical removal of typical carcinoid tumors is a primary treatment for EAS.
  • This intervention can achieve excellent long-term survival and effective control of hypercortisolism.
  • Highlights the importance of identifying and surgically addressing ectopic ACTH-secreting tumors.