Background: Adrenocortical Tumors (ACT) is a rare endocrine malignancy with heterogeneous presentation.
Aim: To evaluate the clinical, biochemical and pathologic characteristics of pediatric ACT in a single tertiary center.
Subjects and methods: Review of 28 medical records with childhood ACT (chronological age (CA) <18 years (y) treated between 1987 and 2017. Clinical, biochemical, and histological features (Wieneke index), staging (ST) according to COG system, and therapeutic interventions were evaluated.
Results: Mean CA at diagnosis was 4.6 y (range 0.317.3 y, median 2.8 y, F/M ratio 2.5/1). Before diagnosis mean duration of symptoms was 10.9 months. Median follow-up was of 3.64 y (range 012 y). Initial clinical signs were hormonal overproduction (virilization, Cushings syndrome) in 57.1%, abdominal mass/pain in 35.7%, and hypertensive encephalopathy in 7.1%. In clinically predominant virilizing ACTs (n=16) mean±S.D. Height-SDS (1.03±1.29) and ΔBACA were significantly higher, while BMI-SDS (0.79±0.8) was significantly lower than in clinically predominant Cushing ACTs (n=10) (P<0.05). Limited disease (ST I/II) was observed in 13 (46.4%), while advanced disease (ST III and IV) was observed in 10 (35.7%) and five patients (17.8%) respectively. Accordingly to hormonal production 3 groups (Gr) were analyzed: Gr1 (n=14) co-secretion of androgens and cortisol, Gr2 (n=8) isolated androgen secretion, and Gr3 (n=2) cortisol secretion (four patients could not be assessed). Very high serum DHEAS levels in Gr1 and Gr2 (X±SD 18 603±16 419 ng/ml) were detected. Serum DHEAS levels were significantly higher in ST IV vs ST I (P=0.03). Total adrenalectomy was performed in 26/28 patients. Eight patients (ST III-IV) received adjuvant chemotherapy (AChemo) with cisplatin, etoposide and doxorrubicin. Disease free survival (DFS) and overall survival (OS) was 100% for ST I-II, and 46.7% and 53.3% for Stage III and IV respectively (media follow-up of 8.4 and 8.6y for DFS and OS). A tendency of higher DFS on AChemo (75%, n=8) vs without AChemo (29%, n=7) was found (OR=0.13, CI 95% 0.011.31). Tumor staging correlated positively and significatively with tumor weight and Wieneke criteria (P<0.01).
Conclusions: We reported the experience in our cohort of 28 pediatric ACT seen in a single center over 30 years. Height-SDS and BMI-SDS mirror ACT hormonal secretion. Very high serum DHEAS levels might be use as a biological marker of tumor stage. Less advanced disease were associated with best patient outcomes. Long term follow-up is needed to draw valid conclusions of using AChemo.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology