Pediatric Adrenocortical Carcinoma: Diagnostic Challenges and Therapeutic Approach — A Case Report

Assia  Haif; Djelloul  Achouri

doi:10.18502/ijpho.v15i4.19637

Assia Haif Department of Pediatric Surgery, University Hospital Center of SETIF, Algeria
Djelloul Achouri Department of Pediatric Surgery, University Hospital Center of SETIF, Algeria

DOI: https://doi.org/10.18502/ijpho.v15i4.19637

Keywords: Adrenocortical Carcinoma, Child, Mortality, Surgical Procedures, Virilism

Abstract

Adrenocortical carcinoma (ACC) is a rare tumor. It constitutes 0.2% of malignant tumors in children and it is considered ten times rarer than in adults. Patients with predisposing syndromes like Li-Fraumeni or Beckwith-Wiedemann may develop these tumors.

Clinical symptoms are dominated by endocrine signs, which are present in 90% of cases. The primary cause is virtualization syndrome, which may occur alone or in conjunction with secondary symptoms caused by hypersecretion of adrenal hormones.

Imaging is indispensable in assessing the malignant nature of the adrenal tumor and its extent. Currently, surgical excision of the tumor is the cornerstone of treatment because its quality often depends on the prognosis of the disease. Adrenocortical carcinoma has a poor prognosis despite treatment, with high chances of recurrence and mortality.