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

Abstract

Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Most oncocytic adrenal tumors are not functional. We present a rare case of right-sided, functional oncocytic adrenocortical adenoma in a 5-year-old boy, who presented with clinical manifestations of precocious puberty and Cushing syndrome. Laparoscopic adrenalectomy showed a well-defined mass weighing 8.4 g and measuring 3 cm in maximum dimension. Histological examination demonstrated no features suggestive of aggressive biological behavior. The patient showed no evidence of recurrent or metastatic disease and continued to have normal serum hormonal levels 28 months following the surgery. In this report, we discuss the clinicopathological characteristics of this rare pathological entity and briefly review the literature on functional oncocytic adrenal tumors in the pediatric population.

Pediatric Adrenocortical Oncocytoma presenting as Cushing’s Syndrome and Peripheral Precocious Puberty: A Case Report and Review of Literature

“…Nine cases of OATs in the pediatric age population were previously reported. [7][8][9][10][11][12][13][14][15] All the patients except one were females, with ages between 3.5 and 16 years. 15 Most tumours involved the right adrenal gland, with sizes ranging from 2.5 to 17.5 cm.…”

“…[7][8][9][10][11][12][13][14][15] All the patients except one were females, with ages between 3.5 and 16 years. 15 Most tumours involved the right adrenal gland, with sizes ranging from 2.5 to 17.5 cm. Six of the cases were diagnosed as adrenocortical adenoma (oncocytoma)…”

“… 7 - 15 All the patients except one were females, with ages between 3.5 and 16 years. 15 Most tumours involved the right adrenal gland, with sizes ranging from 2.5 to 17.5 cm. Six of the cases were diagnosed as adrenocortical adenoma (oncocytoma) on histopathology, while one case was labeled as borderline tumour according to the LWB classification.…”

Cushing’s syndrome due to bilateral oncocytic adrenal tumor

“…Онкоцитомы надпочечников встречаются крайне редко и составляют 1,8% от всех новообразований надпочечников, они обычно доброкачественные и гормонально неактивные, могут быть классифицированы как онкоцитарная аденома, карцинома, опухоль с неопределенным злокачественным потенциалом или феохромоцитома [2]. В литературе описано около 13 клинических случаев односторонних онкоцитом надпочечника, ассоциированных с синдромом Иценко-Кушинга [3][4][5][6][7][8][9][10][11][12][13]. Также в литературе найдено описание одного случая двусторонних гормонально-неактивных онкоцитом надпочечников [14].…”