A Rare Case of Ectopic Cushing's Syndrome

L. Steina, S. Steina, V Pirags, A. Ozolins, J. Gardovskis

Research output: Contribution to journalMeeting Abstractpeer-review


Introduction: Cushing's syndrome has an incidence of 0.7 - 2.4 per million
people per year. In 15% of cases, it’s associated with non-pituitary tumors
secreting ACTH. Pheochromocytoma accounts for 5% of ectopic ACTH
secretion. Aim(s): - Materials and methods: - Results: A 65 year old female
patient was admitted to hospital due to severe muscle and generalized
weakness, shortness of breath and suppurated ulcers. Blood panel showed
glucose levels of 16.3 mmol/L, Na - 140.9 mmol/L, K - 1.5 mmol/L. CT
angiography revealed a PE in the upper lobe of the right lung and a
pathological node 7x6.2 cm in diameter in the left adrenal gland, which was
characteristic of a non-adenoma. ACTH was 152 pg/mL. Catecholamines were
within normal range, and cortisol in 24 hour urine was 3962 nmol/24h. An MRI
of the head showed no signs of a pituitary adenoma. The left adrenal gland
was surgically removed. Pathohistological examination revealed a
pheochromocytoma with a PASS score of 11 points, which proves a malignant
biological potential. Levels of ACTH were evaluated again after the surgery
and were within normal range – 33.2 pg/mL. Conclusion: The most likely
diagnosis after the examination was a malignant ACTH secreting
pheochromocytoma. Data from large series of patients with ectopic ACTH
reveal that the most common cause is a bronchial carcinoid. Our case report
demonstrates utmost importance of interpretation of clinical, biochemical, and
radiological investigation in establishing the correct underlying cause of
Cushing's syndrome.
Original languageEnglish
Article numberQ31
Pages (from-to)319-319
Issue numberSuppl.1
Publication statusPublished - 2017
Event14th Annual ENETS Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease - Barcelona, Spain
Duration: 8 Mar 201710 Mar 2017
Conference number: 14


  • malignant pheochromocytoma

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 3.3. Publications in conference proceedings indexed in Web of Science and/or Scopus database


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