Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Mar-Apr;27(2):157-162.
doi: 10.4103/jiaps.JIAPS_351_20. Epub 2022 Mar 1.

Adrenocortical Neoplasms in Children: Treatment and Outcomes

Vishesh Jain  1 , Mehak Sehgal  1 , Anjan Dhua  1 , Sameer Bakhshi  2 , Devasenathipathy Kandasamy  3 , Kalaivani Mani  4 , Rajni Sharma  5 , Vandana Jain  5 , Nikhil Tandon  6 , Sandeep Agarwala  1
Affiliations

Adrenocortical Neoplasms in Children: Treatment and Outcomes

Vishesh Jain et al. J Indian Assoc Pediatr Surg. 2022 Mar-Apr.

Abstract

Background: Adrenocortical tumors are rare tumors in children with a paucity of outcome data, especially in a resource-challenged setting.

Materials and methods: A retrospective study was conducted to evaluate the presentation, management, and outcomes of children with adrenocortical tumors who presented to our institute from January 1992 to December 2019.

Results: During the study period, 28 children were included. Adenoma was present in nine children and the remaining 19 children had adrenocortical carcinoma. The median age of presentation was 48 months. Nearly half of the children with carcinomas presented with a mass compared to only 10% adenomas (P = 0.049). Virilization was present in 60% of the patients and in most cases was associated with Cushingoid features. Three children (15.7%) had metastasis at presentation. All children underwent surgical excision with a spill in 6 and gross residue in two patients. Recurrence was observed in five patients after a mean duration of 11.8 months. The 4-year overall survival and event-free survival were 100% in patients with adenomas compared to 75% and 44%, respectively, in carcinoma. Children who underwent complete surgical excision without any spill had a relatively favorable outcome.

Conclusion: Adrenocortical tumors are uncommon tumors in children. Adenomas have a favorable outcome. There was no disease-free survival in incompletely resected or metastatic tumors. Tumor spill may also have an adverse outcome in completely resected tumors.

Keywords: Adenoma; Cushing syndrome; adrenalectomy; adrenocortical; adrenocortical carcinoma.

PubMed Disclaimer

Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Presence of only virilizing features in a 4-year-old boy. (b) Presence of cushingoid features in a 3-year-old girl with adrenocortical carcinoma
Figure 2
Figure 2
Kaplan–Meier survival curve of overall survival and event-free survival of patients with adrenocortical carcinoma
See this image and copyright information in PMC

References

    1. Michalkiewicz E, Sandrini R, Figueiredo B, Miranda EC, Caran E, Oliveira-Filho AG, et al. Clinical and outcome characteristics of children with adrenocortical tumors: A report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol. 2004;22:838–45. - PubMed
    1. Ribeiro RC, Pinto EM, Zambetti GP, Rodriguez-Galindo C. The international pediatric adrenocortical tumor registry initiative: Contributions to clinical, biological, and treatment advances in pediatric adrenocortical tumors. Mol Cell Endocrinol. 2012;351:37–43. - PubMed
    1. Ribeiro RC, Figueiredo B. Childhood adrenocortical tumours. Eur J Cancer. 2004;40:1117–26.6. - PubMed
    1. Pinto EM, Rodriguez-Galindo C, Pounds SB, Wang L, Clay MR, Neale G, et al. Identification of clinical and biologic correlates associated with outcome in children with adrenocortical tumors without germline TP53 mutations: A St Jude Adrenocortical Tumor Registry and Children's Oncology Group Study. J Clin Oncol. 2017;35:3956–63–63. - PMC - PubMed
    1. Sabaretnam M, Mishra A, Agarwal G, Agarwal A, Verma AK, Mishra SK. Adrenocortical carcinoma in children and adults: Two decades experience in a single institution. Indian J Cancer. 2016;53:317–21.7. - PubMed