Outcome for Pediatric Adreno-Cortical Tumors Is Best Predicted by the COG Stage and Five-Item Microscopic Score—Report from the German MET Studies

SIMPLE SUMMARY: Pediatric adrenocortical tumors have a poor prognosis. The EXPeRT consortium recently published recommendations for their management. In this study, we report on 161 pediatric ACT patients registered with the German Malignant Endocrine Tumor studies and explore those recommendations. The median age at the diagnosis was 4.3 years, and the mean follow-up was 4.5 years. The 3-year overall (OS) and event-free survival (EFS) estimates were 65.5% and 50.6%. The clinical presentation and prognosis were defined by age. The OS was impaired for patients aged ≥ 4 years, following the initial biopsy, tumor spillage, and incomplete tumor resection, with unfavorable histology, according to the five-item microscopic score and COG stages III and IV. COG stages III and IV and unfavorable histology were impacted as negative prognostic factors upon the EFS and OS. ABSTRACT: Background: Adrenocortical tumors (ACTs) encompassing the adrenocortical adenoma (ACA), carcinoma (ACC), and tumors of undetermined malignant potential (ACx) are rare endocrine neoplasms with a poor prognosis. We report on pediatric ACT patients registered with the Malignant Endocrine Tumor studies and explore the EXPeRT recommendations for management. Patients: Data from the ACT patients (<18 years) were analyzed. For the risk prediction, the patients were retrospectively assigned to the COG stages and the five-item score. Results: By December 2021, 161 patients with ACT (ACA n = 51, ACx n = 19, and ACC n = 91) had been reported (the median age at the diagnosis was 4.3 years with a range of 0.1–17.8), with lymph node and distant metastases in 10.7% and 18.9% of the patients with ACC/ACx. The mean follow-up was 4.5 years (with a range of 0–16.7). The three-year overall (OS) and event-free survival (EFS) rates were 65.5% and 50.6%. In the univariate analyses, the OS was impaired for patients aged ≥ 4 years (p = 0.001) with the initial biopsy (p = 0.016), tumor spillage (p = 0.028), incomplete tumor resection (p < 0.001), unfavorable histology (p = 0.047), and COG stages III/IV (p = 0.002). Multivariate analysis revealed COG stages III/IV and an unfavorable five-item score as independent negative prognostic factors for the EFS and OS. Conclusions: Age defines the clinical presentation and prognosis in pediatric ACTs. The outcome is best predicted by the COG stage and five-item score.