Endocrine Abstracts

Introduction: We report the clinical presentation of pheochromocytoma with Cushing’s syndrome due to ectopic production of ACTH, and assess the histopathological diagnosis, treatment modality and prognostic factors compared with the literature.

Results: Cushing’s Syndrome due to ectopic ACTH production is uncommon and due to pheochromocytoma is extremely rare. We discuss the case of a 50-year-old female who initially presented with vague, non-specific symptoms, such as general and muscle weakness, weight loss, body temperature rise, high blood pressure, increase in fasting blood glucose, in which an ACTH-secreting tumor found to be the cause of her clinical presentation. At admission: height 168 cm, weight 57 kg. asthenic constitution, diffusely hyperpigmented skin, ‘darkened elbow’ symptom, and subcutaneous adipose tissue was insufficiently developed. Laboratory showed AM cortisol of 1488 mmol/l, PM cortisol of 1672 mmol/l, 24 h urinary free cortisol of 3700 nmol/day, AM ACTH level of 178.7 mg /ml, PM ACTH level of 1798 mg /ml and non-suppression of cortisol with overnight dexamethasone suppression test (1 and 8 mg). 24 h urinary level of normetanephrine and metanephrine: normetanephrine – 830 mg/day, metanephrine – 1481 mg/day. Brain MRI showed no pathological changes. CT scan showed tumor of the left adrenal gland (2.7×3, 0×4, 6 cm, density 38H). She underwent 3 weeks therapy by doxazozine and mifepristone before surgery. So, clinical and laboratory signs of Cushing’s syndrome and pheocromocytoma disappeared after left adrenalectomy.

Conclusion: Despite numerous guidelines in pheochromocytoma and Cushing’s syndrome, there are still diagnosis and treatment mistakes due to rarity and complexity of clinical presentation in ACTH-ectopic syndrome caused by pheocromocytoma. So, we need to improve the guidelines for diagnosis and treatment of ACTH-ectopic tumors.