Purpose or Case Report: Pediatric adrenocortical carcinomas (ACCs) are less common than neuroblastomas, but have worse prognosis. Early identification and differentiation from neuroblastomas helps allow prompt management. We describe the imaging features of primary and metastatic ACCs.
Methods & Materials: Retrospective review of 21 histopathologically proven pediatric ACCs was performed. The data reviewed in consensus by two dedicated oncoradiologists on institutional PACS. Clinical information was obtained from EMR, with every histology confirmed by a dedicated oncopathologist.
Results: 21 children (11 girls and 10 boys) were evaluated, with baseline imaging available in 19 patients. Virilization and hirsutism (8/19 each) and Cushingoid features (6/19) were the most common symptoms. TP53 mutation was positive in 5/19 patients, one of which had bilateral ACCs. Mean tumor size amongst the 19 baseline tumors was 7.6 cm (range: 3.2-17.5 cm), with tumors being predominantly well defined, lobulated and heterogeneously hypodense. 10/19 (53%) were solid (>75% solid component), while 9/19 were solid–cystic/necrotic, with 9/19 (47%) having calcifications. 5/19 (26%) demonstrated adjacent organ invasion, with tumor thrombus being present in 8/19 (42%). 14/19 patients were surgically resected, with the other five being inoperable due to locally advanced disease or metastasis at presentation. Overall, 10/21 (48%) patients had metastatic disease, including involvement of lungs (6/10), liver (5/10), and nodes (4/10) (mainly retroperitoneal nodes).
Conclusions: ACCs present with distinct clinical and imaging features, with tumor thrombus, adjacent organ invasion and calcifications. Approximately half the patients develop metastatic disease, making early diagnosis and complete resection important for better outcomes.