Endocrine Abstracts

Introduction: Ectopic Cushing’s syndrome (ECS) is due to ACTH secretion of no pituitary neuroendocrine tumors and represents around 5–10% of all cases of ACTH dependent Cushing’s syndromes.

Methods: Six cases of ECS diagnosticated from 2008 to 2017 were studied. Patients’ age ranges from 36 to 69 years (mean 53), four females and two males. Three had pancreatic tumors, two small cell lung carcinomas and one pheocromocytoma. At diagnosis, in 83% disease was disseminated. Hypercortisolism was diagnosed before neoplasic process in five cases. All patients presented rapid clinical evolution except for patient with pheocromocytoma. Weakness, hyperpigmentation, edema, hypertension and diabetes mellitus were the most important signs and symptoms. two patients presented severe psychotic disorders. Hypokaliemia was observed in five cases (mean potassium level 2.35 mEq/l) and metabolic alkalosis in 4. Bioquimical parameters were: cortisol 73 μg/dl (29.2–136.6), ACTH 561.1 pg/ml (46–1884), urinary cortisol 6596.9 μg/24 h (226–2,3247), nugent 60.5 μg/dl (26.9–135). All patients were treated to control the hypercortisolism, one with ketoconazole, two with metopirone, two with combination of ketoconazole and metopirone and one with mifepristone. No bilateral adrenalectomy were performed, only one adrenal embolectomy. Surgical removal of primary tumor was performed in two pancreatic tumors and pheocromocytoma, and four were treated with chemotherapy. Five patients died few months after diagnosis, pheocromocytoma is cured and one patient with lung carcinoma is under control but is suffering tumor progression.

Conclusions: Any neuroendocrine tumor may be associated with ECS. Survival depends on primary tumor histology, presence of metastases and severity of hypercortisolism. This is why a multidisciplinary approach is required for the correct diagnostic and therapeutic management.