Endocrine Abstracts

Introduction: Aggressive ACTH-independent Cushing’s syndrome is rare and frequently related to malignant adrenocortical tumor. However, in exceptional situations, ectopic steroids secretion has been described.

Case report: We report the case of an 87 years-old woman referred to our clinic in September 2020 after a low trauma sacral fracture. Her recent medical history revealed the onset, in the previous months, of severe hypokalemia, uncontrolled type 2 diabetes (glycated hemoglobin 9.2%), worsening of arterial hypertension and spontaneous bruising. Because of the medical history, a Cushing’s syndrome (CS) was suspected and after exclusion of exogenous glucocorticoid use, specific tests were carried out. Laboratory findings confirmed the diagnosis of CS: 24-hour urine free cortisol >4×ULN, cortisol after 1-mg overnight dexamethasone suppression test of 58.8 μg/dl, undetectable adrenocorticotropic hormone (ACTH) levels, and hypokalemia. The subsequent abdominal CT scan revealed normal adrenal glands, but showed a suspicious 8-cm pelvic mass, which was suggestive of malignancy. Unfortunately, given the old age and the severe clinical picture, no further investigations were feasible and a medical therapy with metyrapone was initiated. After few weeks of therapy, we observed an improvement of glucose and pressure control and the restoration of normal potassium levels, even though the clinical picture remained severe. After six months a clinical improvement was observed, and the patient could undergo an abdomen MRI for better characterizing the pelvic mass. This second radiologic evaluation showed a significant increase of the pelvic mass diameter, which had doubled, reaching the diameter of 17 centimeters. Because of the rapid tumor growth and the presence of ACTH independent hypercortisolism, the patients could be suspected to be affected by an ectopic cortisol secreting adrenocortical carcinoma. Histological confirmation would be necessary, but surgical options or other invasive procedures are not feasible.

Discussion: Ectopic adrenal carcinomas may result from adrenal embryonic remnants. Adrenocortical neoplasms arising in ectopic locations are extremely rare, and cortisol-secreting ones are even rarer, with only a few cases reported in the literature. The treatment of ectopic adrenocortical carcinoma does not differ from that of eutopic tumors. However, ectopic adrenocortical carcinomas are, in general, aggressive and poorly differentiated and they are, therefore, associated with a high mortality rate.