Pediatric Adrenocortical Oncocytoma presenting as Cushing’s Syndrome and Peripheral Precocious Puberty
A Case Report and Review of Literature
DOI:
https://doi.org/10.15605/jafes.036.02.17Keywords:
adrenal oncocytoma, functioning adrenal adenoma, pediatric adrenal tumoursAbstract
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.
Downloads
References
Costanzo PR, Paissan AL, Knoblovits P. Functional plurihormonal adrenal oncocytoma: Case report and literature review. Clin Case Rep. 2017;6(1):37-44. https://pubmed.ncbi.nlm.nih.gov/29375834. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5771899. https://doi.org/10.1002/ccr3.1279.
Mearini L, Del Sordo R, Costantini E, Nunzi E, Porena M. Adrenal oncocytic neoplasm: A systematic review. Urol Int. 2013;91(2):125-33. https://pubmed.ncbi.nlm.nih.gov/23147196. https://doi.org/10.1159/000345141.
Bisceglia M, Ben-Dor D, Pasquinelli G. Oncocytic adrenocortical tumors. Pathol Case Rev. 2005;10(5):228-42. https://doi.org/10.1097/01.pcr.0000175102.22075.54.
Renaudin K, Smati S, Wargny M, et al. Clinicopathological description of 43 oncocytic adrenocortical tumors: Importance of Ki-67 in histoprognostic evaluation. Mod Pathol. 2018;31(11):1708–16. https://pubmed.ncbi.nlm.nih.gov/29921900. https://doi.org/10.1038/s41379-018-0077-8.
Kakimoto S, Yushita Y, Sanefuji T, et al. Non-hormonal adrenocortical adenoma with oncocytoma-like appearances. Hinyokika Kiyo. 1986;32(5):757-63. https://pubmed.ncbi.nlm.nih.gov/3751804.
Wong DD, Spagnolo DV, Bisceglia M, Havlat M, McCallum D, Platten MA. Oncocytic adrenocortical neoplasms—A clinicopathologic study of 13 new cases emphasizing the importance of their recognition. Hum Pathol. 2011;42(4):489-99. https://pubmed.ncbi.nlm.nih.gov/21237489. https://doi.org/10.1016/j.humpath.2010.08.010.
Gumy-Pause F, Bongiovanni M, Wildhaber B, Jenkins JJ, Chardot C, Ozsahin H. Adrenocortical oncocytoma in a child. Pediatr Blood Cancer. 2008;50(3):718-21. https://pubmed.ncbi.nlm.nih.gov/17091483. https://doi.org/10.1002/pbc.21090.
Tahar GT, Nejib KN, Sadok SS, Rachid LM. Adrenocortical oncocytoma: A case report and review of literature. J Pediatr Surg. 2008;43(5):E1-3. https://pubmed.ncbi.nlm.nih.gov/18485928. https://doi.org/10.1016/j.jpedsurg.2007.12.067 .
Lim Y-J, Lee S-M, Shin J-H, Koh H-C, Lee Y-H. Virilizing adrenocortical oncocytoma in a child: A case report. J Korean Med Sci. 2010;25(7):1077-9. https://pubmed.ncbi.nlm.nih.gov/20592902. PMCID: PMC2890887. https://doi.org/10.3346/jkms.2010.25.7.1077.
Subbiah S, Nahar U, Samujh R, Bhansali A. Heterosexual precocity: Rare manifestation of virilizing adrenocortical oncocytoma. Ann Saudi Med. 2013;33(3):294-7. https://pubmed.ncbi.nlm.nih.gov/23793435. PMCID: PMC6078526. https://doi.org/10.5144/0256-4947.2013.294.
Sharma D, Sharma S, Jhobta A, Sood RG. Virilizing adrenal oncocytoma. J Clin Imaging Sci. 2012;2:76. https://pubmed.ncbi.nlm.nih.gov/23393632. PMCID: PMC3551489. https://doi.org/10.4103/2156-7514.104309.
Pereira BD, Rios ES, Cabrera RA, Portugal J, Raimundo L. Adrenocortical oncocytoma presenting as Cushing’s syndrome: An additional report of a paediatric case. Endocr Pathol. 2014;25(4):397-403. https://pubmed.ncbi.nlm.nih.gov/25077961. https://doi.org/10.1007/s12022-014-9325-8.
Kawahara Y, Morimoto A, Onoue A, Kashii Y, Fukushima N, Gunji Y. Persistent fever and weight loss due to an interleukin-6-producing adrenocortical oncocytoma in a girl—Review of the literature. Eur J Pediatr. 2014;173(8):1107-10. https://pubmed.ncbi.nlm.nih.gov/24610396. https://doi.org/10.1007/s00431-014-2292-8.
Yordanova G, Iotova V, Kalchev K, et al. Virilizing adrenal oncocytoma in a 9-year-old girl: Rare neoplasm with an intriguing postoperative course. J Pediatr Endocrinol Metab. 2014;28(5-6):685-90. https://pubmed.ncbi.nlm.nih.gov/25514324. https://doi.org/10.1515/jpem-2014-0308.
Al Badi MK, Al-Alwan I, Al-Dubayee M, et al. Testosterone- and cortisol-secreting oncocytic adrenocortical adenoma in the pediatric age-group. Pediatr Dev Pathol. 2018;21(6):568–73. https://pubmed.ncbi.nlm.nih.gov/29426273. https://doi.org/10.1177/1093526617753045.
Published
How to Cite
Issue
Section
License
Copyright (c) 2021 Niya Narayanan, Sadishkumar Kamalanathan, Jayaprakash Sahoo, Dukhabandhu Naik, Sambandan Kumaravel , Rajesh Nachiappa Ganesh
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Journal of the ASEAN Federation of Endocrine Societies is licensed under a Creative Commons Attribution-NonCommercial 4.0 International. (full license at this link: http://creativecommons.org/licenses/by-nc/3.0/legalcode).
To obtain permission to translate/reproduce or download articles or use images FOR COMMERCIAL REUSE/BUSINESS PURPOSES from the Journal of the ASEAN Federation of Endocrine Societies, kindly fill in the Permission Request for Use of Copyrighted Material and return as PDF file to jafes@asia.com or jafes.editor@gmail.com.
A written agreement shall be emailed to the requester should permission be granted.