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 and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. 2018 Jan 01 [Epub ahead of print]
Maryam K Al Badi, Ibrahim Al-Alwan, Mohammed Al-Dubayee, Ayed Al-Anzi, Maram S Al Turki, Noura Aloudah, Khaled O Alsaad
1 Department of Pediatrics, 48168 King Abdulaziz Medical City , Riyadh, Saudi Arabia., 4 Department of Pediatrics, Al Jahra Hospital, Kuwait City, Kuwait., 3 College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.