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Published on: January 17, 2018
Adrenocortical Neoplasms in Children: Treatment and Outcomes
Vishesh Jain1, Mehak Sehgal1, Anjan Dhua1
1Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
Insights
Pediatric adrenocortical tumors are rare. While adenomas have good outcomes, adrenocortical carcinomas require complete surgical resection to improve survival rates and prevent recurrence.
Area of Science:
- Pediatric Oncology
- Endocrinology
- Surgical Oncology
Background:
- Adrenocortical tumors are rare in children, with limited outcome data, particularly in resource-limited settings.
- Understanding the characteristics and outcomes of these rare pediatric tumors is crucial for improving patient management.
Purpose of the Study:
- To evaluate the clinical presentation, management strategies, and treatment outcomes of pediatric adrenocortical tumors.
- To analyze factors influencing survival and recurrence in children diagnosed with adrenocortical tumors.
Main Methods:
- Retrospective study of 28 children with adrenocortical tumors treated between January 1992 and December 2019.
- Data collected on tumor type (adenoma vs. carcinoma), clinical presentation, surgical management, and patient outcomes.
Main Results:
- Of 28 children, 9 had adenomas and 19 had adrenocortical carcinomas. Carcinomas were more frequently associated with presenting masses (P=0.049).
- Virilization and Cushingoid features were common. Metastasis occurred in 15.7% at presentation. Recurrence was noted in 5 patients.
- Four-year survival was 100% for adenomas versus 75% overall and 44% event-free for carcinomas. Complete resection without tumor spill correlated with better outcomes.
Conclusions:
- Pediatric adrenocortical adenomas have a favorable prognosis.
- Complete surgical excision is critical for managing adrenocortical carcinoma, as incomplete resection or tumor spill is associated with poor outcomes and no disease-free survival.
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.
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