Abstract

Adrenocortical oncocytic neoplasms (oncocytomas) are extremely rare; only approximately 159 cases have been described so far. The majority are nonfunctional and benign. We describe an unusual case of a functional oncocytoma secreting an excess of glucocorticoids (cortisol) and androgens (androstenedione and DHEAS), a pattern of plurihormonal cosecretion previously not reported in men, presenting with endocrine manifestations of Cushing's syndrome. The neoplasm was considered to be of uncertain malignant potential (borderline) according to the Lin-Weiss-Bisceglia criteria.

Highlights

  • Adrenocortical oncocytic neoplasms are very rare tumours

  • We describe an unusual case of a functional oncocytoma secreting an excess of glucocorticoids and androgens, a pattern of plurihormonal cosecretion previously not reported in men, presenting with endocrine manifestations of Cushing’s syndrome

  • This patient presented with an exceptional adrenocortical oncocytoma of uncertain malignant potential, according to the Lin-Weiss-Bisceglia criteria for the specific assessment of the malignant potential of oncocytic adrenocortical neoplasms [4], cosecreting an excess of cortisol, DHEAS, and androstenedione

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Summary

Background

Adrenocortical oncocytic neoplasms (oncocytomas) are very rare tumours. Since the first description by Kakimoto et al [1] in 1986, only 159 cases have been reported [2], and they are usually nonfunctional and benign [3]. 17% of the adrenal oncocytomas are functional [3]. We report a rare case of a functional adrenocortical oncocytoma cosecreting cortisol, androstenedione, and DHEAS

Case Presentation
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