Endocrine Abstracts

Background: Cushing’s syndrome is represented by the cummulation of signs and symptoms of excess glucocorticoids and has many potential causes. Approximately 20 percent of all cases are represented by ACTH-independent adrenal tumors – with a majority of these being represented by cortisol secreting adrenal adenomas. Generally, a large adrenal tumor has over 5 cm in diameter and the risk of it being malignant raises proportionately with the dimensions. Large adrenal tumors are rare and the management and surgical indication is patient-specific.

Case report: A 20 years old female presented for weight gain (30 kg in the last 3 years), bradimenorrhea, hirsutism and treatment-resistant acne, referred by a dermatologist. Clinical exam revealed general obesity with little central redistribution, facial and shoulders pustular acne, vessel fragility (bruises and hematomas), purple axillary striae and hirsutism (Ferriman score=29). Blood pressure was normal upon multiple examinations. The patient had upper-normal 0800 h cortisol values (19.19 μg/dl) with suppressed 0800 h plasma ACTH (1.05 pg/ml), with loss of circadian secretion rhythm (late-night serum cortisol – 15.8 μg/dl) and lack of cortisol suppression both after 1 mg overnight dexamethasone (17.8 μg/dl) and after 2 mg×2 days dexamethasone suppression test (19.7 μg/dl). She also had increased free urinary cortisol levels (324 μg/24 h) and late-night salivary cortisol (19.1 nmol/l). She also had increased androgens (testosterone and androstenedione), with a FAI of 14, with upper-normal values of DHEA-S, without suppression after the dexamethasone dynamic tests. Glucose metabolism was normal, with normal HOMA-IR score. Abdominal MRI was performed, revealing a large left adrenal tumor 6.7/5.6/6.3 cm and a hypo-plastic right adrenal gland. The diagnosis was ACTH-independent Cushing syndrome due to adrenal tumor secreting cortisol and androgens, with radical-surgery indication.

Treatment: Taking into account the potential malignancy, the patient underwent open surgery. The histopathological examination revealed an 8/7/7 cm cortical adrenal adenoma, without any malignancy characteristics. The immunohistochemistry analysis is pending.

Follow-up: Postoperatively, the patient showed adrenal insufficiency. Five months later, the basal cortisol value was low (0.97 μg/dl) with a slighly raised value of ACTH (56.64 pg/ml). The MRI confirmed total removal of the left adrenal tumor and a slightly smaller right adrenal gland.

Conclusion: Despite having a large adrenal tumor, our young patient had a paucisymptomtic ACTH – independent Cushing syndrome. Morning cortisol values aren’t enough to exclude hypercortisolism especially in young patients.