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Ectopic ACTH syndrome. Diagnostic and therapeutic aspects
American Journal of Surgery
|February 1, 1985
Summary
Adrenocorticotropic hormone (ACTH)-secreting tumors outside the pituitary and adrenal glands can cause Cushing's syndrome. Surgical removal of nonmalignant tumors offers an excellent prognosis, while adrenalectomy benefits those with malignancy.
Area of Science:
- Endocrinology
- Oncology
- Surgical Pathology
Background:
- Ectopic ACTH syndrome arises from tumors outside the pituitary and adrenal glands.
- Clinical manifestations include hypertension, edema, and cushingoid appearance.
- Biochemical hallmarks include elevated cortisol and ACTH levels.
Purpose of the Study:
- To review clinical and biochemical features of non-pituitary, nonadrenal ACTH-secreting tumors.
- To evaluate the efficacy of different surgical interventions.
- To determine prognostic factors for patient outcomes.
Main Methods:
- Retrospective analysis of 25 patients with ACTH-secreting tumors.
- Review of clinical presentations, biochemical data, and treatment modalities.
- Assessment of surgical outcomes including adrenalectomy, tumor excision, and hypophysectomy.
Main Results:
- Common tumor sources included bronchial carcinoid and small cell carcinoma.
- Elevated urinary free cortisol was universal; hypokalemia and elevated ACTH were frequent.
- Bilateral adrenalectomy was performed in 56% of patients, tumor excision in 36%.
Conclusions:
- Bilateral adrenalectomy is beneficial for managing symptoms and prolonging survival in patients with ectopic ACTH syndrome, especially with slow-growing or unknown malignancies.
- Excision of nonmalignant ACTH-producing tumors leads to an excellent long-term prognosis.