Endocrine Abstracts

Cushing’s syndrome caused by ectopic ACTH secretion (EAS) constitutes ~10% of Cushing’s syndrome (CS).

The aim of this study was to present experience of our Clinic with EAS.).

Patients: Twelve patients, aged 14–70 years: four females and eight males.

Outcomes and measurements: Clinical features, medical examination, morning electrolytes, glucose level, serum cortisol, ACTH levels, midnight plasma cortisol concentration and dexamethasone suppression tests. Imaging tests: computed tomography (CT), somatostatin receptor scintigraphy (SRS), magnetic resonance imaging, positron emission tomography and histopathological examinations.

Results: Seven patients were diagnosed as NEN, one patient as medullary thyroid carcinoma (MTC), one patient as pheochromocytoma, one patient as ovarian carcinoma, one patient as lung carcinoma and one as esthesioneuroblastoma.

Clinical features, like those in CS: central obesity, signs of hypercatabolism, bruising and severe amyotrophy with or without facial fullness or weight gain, severe hypokalemia, life-threatening infections, muscular weakness and psychiatric disorders were present in almost all patients. Flush or diarrhea were observed in patients with MTC and with metastatic carcinoid tumors.

Enlarged adrenal glands were detected in CT scans in all patients. SRS was positive in all patients with NET and carcinoids.

Because of severe condition and complications each patient required individual treatment.

Ten patients died, eight due to metastases and two due to metabolic complications.

Conclusion: i) Localization of the ACTH-secreting tumor is often difficult to dicover and may require multiple modalities.

ii) Surgical resection of the source of ACTH production may be curative in cases of benign neoplasms.

iii) Metastatic tumors as well as metabolic complication of hypercortisolemia can be the reason of patient’s death.