Cushing's Syndrome Due to Ectopic ACTH Production of Pheochromocytoma. Case Report

Presentation Number: SUN-0780
Date of Presentation: June 22nd, 2014

Vadim Krylov*1, Ekaterina Dobreva2, Nikolay Kuznetsov2, Nona Vadimovna Latkina2 and Eugenia Marova2
1University Clinical Hospital – 1, I.M. Sechenov First Moscow State Medical University, Moscow, Russia, 2Endocrinology Research Centre, Moscow, Russia


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.
Case report: 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-hour urinary free cortisol of 3700 nmol/day, AM ACTH level of 178.7 mg /ml, PM ACTH level of 179,8 mg /ml and non-suppression of cortisol with overnight dexamethasone suppression test (1 mg and 8 mg). 24-hour 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 x3,0x4,6 cm, density 38H). She underwent two weeks therapy by doxazozine before surgery. So, clinical and laboratory signs of Cushing syndrome and pheocromocytoma disappeared after left adrenalectomy.
Conclusion: Despite numerous guidelines in Pheochromocytoma and Cushing 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.


Nothing to Disclose: VK, ED, NK, NVL, EM