Adrenocortical oncocytic neoplasm: A systematic review

A case of adrenocortical oncocytic carcinoma arising in ectopic adrenal tissue: a multidisciplinary diagnostic challenge

“…Unlike most adrenocortical malignancies, the size of the mass and the imaging findings on CT or MRI are not pathognomonic for AOC. Although the percentage washout on CT has been correlated to the differentiation of benign versus malignant AON [3], this finding does not have proven statistical significance and requires a high index of preoperative suspicion to draw a conclusion of AON amongst other adrenal tumours on CT. Fine needle aspiration and core biopsies are seldom helpful to define AON preoperatively [3,4].…”

“…AONs have a reported recurrence rate of 16%, with most recurrences occurring within first 5 years [3]. There is no specific surveillance schedule for AONs, and most patients are followed according to a standard protocol for ACC.…”

“…Most AONs are nonfunctional and, therefore, are discovered incidentally or present with vague abdominal or back pain. AONs are diagnosed predominantly in females with an average age of 44 years at diagnosis [3]. We report a case of a 26-year-old female who presented with vague leftsided abdominal pain and a large left retroperitoneal mass on CT, which was eventually diagnosed as extra-adrenal adrenocortical oncocytic carcinoma (AOC).…”

ACTH-Independent Cushing’s Syndrome Associated with Left Adrenocortical Oncocytoma of Uncertain Malignant Potential

“…Functioning ACONs are reported with a frequency of 17–34%, of which less than 6% are cortisol-secreting. Moreover, although 41% of ACONs are of uncertain malignant potential, the functioning forms of these range between 22 and 29% [ 5 , 8 ].…”

“…These tumours are usually incidentalomas, unwittingly detected during radiological investigations performed for other medical reasons. Nevertheless, up to 35% are capable of synthesizing hormones such as aldosterone and cortisol, originating visible clinical manifestations [ 5 ]. Indeed, the virilisation and feminization syndromes due to sexual hormone hypersecretion are well described in the literature, as well as Cushing's and Conn's syndromes due to primary hypercortisolism and hyperaldosteronism, respectively.…”