Endocrine Abstracts

Introduction: Pheochromocytomas are neuroendocrine tumors derived from chromaffin cells which secrete catecholamines. Cushing syndrome due to ACTH-secreting pheochromocytomas is a rare condition and early recognition and treatment is important to decrease morbidity and mortality.

Case presentation: We present the case of an 53 years-old man who arrived at the hospital for pain in the right scapula agravated in the last three months with iradiation in the right flanc, heart palpitations, headache and weight loss of 10 kilograms in the last 6 months. General examination revealed an abdomen without signs of peritoneal irritation, painful in the right flanc, high blood pressure (232/165 mmHg) and tachycardia (128/bpm). Blood tests showed leukocytosis (20 600/mm³), mild normocytic normochromic anemia with hemoglobine at 10.8 g/dl, hyperglycemia (225 mg/dl) and slightly elevated liver enzymes (ASAT = 94 UI/I, ALAT = 92 UI/I, GGT = 197 UI/I). The computed tomography revealed a large solid adrenal mass measuring 100 mm in the largest diameter with an important vascularization. The patient was trasferred to our department, where he underwent further biochemical testing to establish the functional status of the tumor. Blood tests showed high values for plasma normetanephrines (56.16 nmol/l), urinary normetanephrines (109.39 μmol/24 H), plasma cortisol (2 270 nmol/l), free urinary cortisol (161 nmol/24 H), plasma ACTH level at 22 pg/ml, normal values for 11-deoxycortisol, total testosterone, DHEAS and aldosterone-renin ratio and slightly elevated values for plasma metanephrines (0.54 nmol/l) and urinary metanephrines (2.54 μmol/24 H). Considering the 50 times higher values of plasma and urinary normetanephrines and 2 times higher values of plasma and urinary metanephrines, the diagnosis of pheochromocytoma was established which also associated a secretion of cortisol. A fluorodeoxyglucose-positron emission tomography was performed before the intervention to exclude possible secondary determinations, which revealed an uptake only at the level of the right adrenal tumor. The treatment with alfa and beta blockers was started and the patient was sent to the surgery department for the excision of the adrenal mass. Post intervention the cortisol and the catecholamine values returned to normal.

Conclusion: Considering the ACTH values higher than 20 pg/ml with very high cortisol values before the surgical intervention and the normalization of cortisolemia after the extirpation of the adrenal tumor, we have considered that it was a Cushing syndrome due to ACTH-secreting pheochromocytoma.