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Ectopic ACTH Syndrome

A paraneoplastic syndrome caused by ACTH release from non-pituitary tumours.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Ectopic ACTH Syndrome?

Ectopic ACTH syndrome is a paraneoplastic form of Cushing syndrome that develops with excessive ACTH or CRH secretion from non-pituitary neuroendocrine tumours. The most common causes are small-cell carcinoma of the lung, bronchial carcinoid, medullary thyroid carcinoma, phaeochromocytoma, thymic carcinoid and pancreatic neuroendocrine tumours.

The disease has a rapid course; the classic Cushing findings may not be prominent. Hypokalaemic alkalosis, hypertension, diabetes, muscle weakness, peripheral oedema and hyperpigmentation are predominant. Pigmentation is striking due to very high ACTH levels.

In diagnosis, high ACTH and cortisol, lack of suppression with high-dose dexamethasone, and localisation of the primary tumour with imaging methods such as CT/MRI and Ga-68 DOTATATE PET/CT are important. Treatment is resection of the primary tumour and medical cortisol suppression.

Symptoms

Rapidly progressive muscle weakness
Marked hyperpigmentation
Severe hypertension
Hypokalaemic metabolic alkalosis
Decompensated diabetes
Peripheral oedema
Psychiatric disturbances
Weight loss (in contrast to classical Cushing)

Risk Factors

Smoking (small-cell lung cancer)
Endocrine tumour syndromes (MEN 1)
History of bronchial carcinoid
Medullary thyroid carcinoma
Thymic neuroendocrine tumours
Phaeochromocytoma
Pancreatic neuroendocrine tumours
Chronic lung diseases

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Rapidly developing hypokalaemia and hypertension
  • Marked hyperpigmentation
  • Unexplained decompensated diabetes
  • Proximal muscle weakness
  • Hypercortisolism with a lung mass
  • Suspicion of paraneoplastic syndrome

Treatment Methods

01
Surgical resection of the primary tumour
02
Steroidogenesis inhibitors (ketoconazole, metyrapone)
03
Bilateral adrenalectomy (refractory cases)
04
Chemotherapy or radiotherapy (depending on tumour)
05
Potassium and electrolyte replacement
06
Antihypertensive treatment
07
Diabetes management
08
Multidisciplinary oncology and endocrinology follow-up

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.