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Pancreatic ACTH Hypersecretion and Pituitary Macroadenoma
Chiara M Bettale1, Jason W Allen2,3, Zaid K Mahdi4
1Emory University School of Medicine, Atlanta, GA 30322, USA.
JCEM Case Reports
|November 1, 2023
Summary
A rare pancreatic neuroendocrine tumor caused Cushing syndrome by secreting ACTH, leading to hypertensive emergency and myocardial infarction. Surgical removal resolved symptoms, highlighting the need for thorough endocrine testing.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- Cushing syndrome is characterized by excessive cortisol exposure.
- Ectopic ACTH secretion can mimic pituitary-dependent Cushing disease.
Observation:
- A patient presented with hypertensive emergency, myocardial infarction, and symptoms of Cushing syndrome.
- Elevated urinary/salivary cortisol and ACTH levels were noted, alongside a pituitary macroadenoma.
- A pancreatic mass was identified, confirmed as an ACTH-secreting neuroendocrine tumor.
Findings:
- The pancreatic neuroendocrine tumor was the source of ectopic ACTH secretion.
- Surgical resection of the pancreatic tumor led to resolution of Cushing manifestations.
- Post-operative management included temporary hydrocortisone replacement therapy.
Implications:
- This case underscores the importance of dynamic endocrine testing for diagnosing Cushing syndrome.
- It highlights the rare coexistence of ectopic ACTH-secreting tumors and pituitary macroadenomas.
- Accurate diagnosis and localization of the ACTH source are critical for effective treatment.
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