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

Niya Narayanan; Sadishkumar Kamalanathan; Jayaprakash Sahoo; Dukhabandhu Naik; Sambandan Kumaravel; Rajesh Nachiappa Ganesh

doi:10.15605/jafes.036.02.17

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

J ASEAN Fed Endocr Soc. 2021;36(2):205-208. doi: 10.15605/jafes.036.02.17. Epub 2021 Oct 6.

Authors

Niya Narayanan¹, Sadishkumar Kamalanathan¹, Jayaprakash Sahoo¹, Dukhabandhu Naik¹, Sambandan Kumaravel², Rajesh Nachiappa Ganesh³

Affiliations

¹ Department of Endocrinology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
² Department of Pediatric Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
³ Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.

Abstract

Oncocytic adrenocortical tumours (OATs) or oncocytomas are extremely rare and are usually benign and nonfunctional. We report the case of a 4-year-old male with a right-sided, functional oncocytic adrenocortical adenoma, who presented with precocious puberty and Cushing's syndrome. After work-up, the patient underwent laparoscopic adrenalectomy. The excised adrenal mass weighed 21 g and measured 3.5 cm in maximum dimension. Histological examination demonstrated no features suggestive of aggressive biological behaviour. The patient had no features of recurrent or metastatic disease and had prepubertal testosterone levels with suppressed hypothalamic-pituitary-adrenal axis twelve months after the surgery. A discussion of this case and a review of the literature on functional OATs in the pediatric population are presented.

Keywords: adrenal oncocytoma; functioning adrenal adenoma; pediatric adrenal tumours.

Publication types

Case Reports