Adrenocortical Oncocytoma Presenting as Cushing’s Syndrome: An Additional Report of a Paediatric Case

Abstract

Oncocytomas are tumours predominantly or exclusively composed of oncocytes, cells with granular and eosinophilic cytoplasm filled with mitochondria. Although they can occur in every organ, they are rare in adrenal glands, and in paediatric patients they are even rarer, with only three case reports previously published. We present a preschool child developing Cushing's syndrome due to an adrenocortical oncocytoma, which was confirmed immunohistochemically with antibodies to the mitochondrial electron complex 2. A 5.8-year-old girl presented with clinical features of Cushing's syndrome. ACTH-independent hypercortisolism was confirmed biochemically and a left adrenal mass was detected by imaging and removed by laparotomy. Histopathological analysis revealed a tumour composed of more than 95 % of oncocytes, confirmed immunohistochemically with antibodies to subunits A and B of the mitochondrial enzyme succinate dehydrogenase. Using the Lin-Weiss-Bisceglia score system and the reticulin algorithm, this tumour was categorized as a benign adrenocortical oncocytoma. The patient currently has 64 months of follow-up, without any evidence of relapse of symptoms. To our knowledge, we herein present the youngest patient developing an adrenocortical oncocytoma and the first manifestation of Cushing's syndrome due to this rare neoplasm in paediatric patients. We also emphasize the clinical usefulness of immunohistochemistry to the mitochondrial enzyme succinate dehydrogenase to confirm the oxyphilic nature of adrenocortical oncocytomas.

Functional plurihormonal adrenal oncocytoma: case report and literature review

“…Recommended tests include measurement of plasma or urinary fractionated metanephrines, low‐dose dexamethasone suppression test, measurement of ACTH, estradiol, androgens (DHEA‐S, delta 4 androstenedione, 17 hydroxyprogesterone, and testosterone) aldosterone and plasma renin activity. In our review of the literature, approximately one‐third of benign oncocytomas were functional, secreting androgen in six cases , androgens and cortisol in five cases , cortisol in six cases , estradiol in two cases , IL‐6 in two cases , aldosterone in four cases , and catecholamines in the remaining case . Among borderline tumors or tumors of uncertain malignant potential, hormonal hypersecretion was found in 17% of cases: four androgen‐secreting tumors , two corticosteroid‐secreting tumors , two catecholamine‐secreting tumors , one corticosteroid‐ and androgen‐secreting tumor , one aldosterone‐secreting tumor , and one 17‐ketosteroids‐secreting tumor .…”

“…In most cases, these tumors were detected incidentally, while in 18.5% of cases patients presented with abdominal pain . Other less frequent forms of presentation were hirsutism , virilization , pseudoprecocious puberty , Cushing's syndrome , blood hypertension , fever , pain in lower limbs , fatigue , and feminization .…”

Testosterone- and Cortisol-secreting Oncocytic Adrenocortical Adenoma in the Pediatric Age-group

“…4,8 Occasionally, OAT can be associated with clinical manifestations related to excess sex and/or steroid and hormonal production such as virilization, feminization, and Cushing's syndrome. 6,11 Adrenocortical tumors are particularly rare in children and adolescents and account for less than 0.2% of all pediatric neoplasms. 6 Only 7 cases of functional OATs in the pediatric age population were previously reported.…”

Pediatric Adrenocortical Tumors: Clinicopathological Features—An Update

“…2A, B). [24][25][26][27][28][29] The extreme rarity of pediatric oncocytic ACTs makes it difficult to predict their biological behavior. Unlike conventional (nononcocytic) ACTs, there is no general consensus about the pathological scoring system which should be applied in pediatric oncocytic ACTs.…”

“…Clinically, four cases presented high levels of sex hormones and signs of virilization or pseudoprecocious puberty, one case presented with a Cushing syndrome, and one case with fever and weight loss due to interleukin-6 production. [24][25][26][27][28][29] Based on the Lin-Weiss-Bisceglia scoring system, five cases were classified as benign (adrenocortical oncocytomas) and the remaining case as "borderline tumor." 27 Notably, in our experience we have encountered only one case of oncocytic ACT in a 7-year-old boy with pseudoprecocious puberty.…”