Abstract
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.
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 language | English |
|---|---|
| Article number | Q31 |
| Pages (from-to) | 319-319 |
| Journal | Neuroendocrinology |
| Volume | 105 |
| Issue number | Suppl.1 |
| Publication status | Published - 2017 |
| Event | 14th Annual ENETS Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease - Barcelona, Spain Duration: 8 Mar 2017 → 10 Mar 2017 Conference number: 14 https://pubmed.ncbi.nlm.nih.gov/29069642/ https://www.karger.com/Book/Home/276889 |
Keywords*
- 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