Pediatric adrenocortical tumors are rare and heterogeneous endocrine malignancies.
To report clinical, biochemical, and histological features, staging, and therapeutic interventions in a cohort of 28 patients treated at a single tertiary center.
A retrospective review of medical records of children with PACT (diagnosed before <18 years of age) followed between 1987–2018 at Hospital de Pediatría Garrahan, Buenos Aires, Argentina.
Mean age at diagnosis was 4.6 years (range, 0.3–17.3 years) and median follow-up was 4.17 years (range, 0–12 years). Female to male ratio was 2.5:1. Signs and symptoms that prompted medical intervention were hormonal overproduction (57%), abdominal complaints (36%), and hypertensive encephalopathy (7%). In patients with clinically virilizing tumors (n=16) mean height standard deviation score (SDS) and bone age advance were significantly higher while body mass index (BMI) SDS was significantly lower than in those with clinical Cushing’s (n=10) (p<0.05). Serum dehydroepiandrosterone sulfate (DHEAS) levels were significantly higher in stage IV than in stage I (p=0.03). Total adrenalectomy was performed in 26 patients. Eight patients (stage III-IV) received adjuvant chemotherapy. Five-year overall and disease-free survival were 100% for ST I-II, and 51% (95% CI 21–82) and 33% (95% CI 1.2–65) for ST III-IV, respectively (p=0.002). No statistical difference was found when comparing 2-year parameters with and without adjuvant chemotherapy.
Height SDS and BMI SDS seem to mirror hormonal secretion in pediatric adrenocortical tumors. Higher DHEAS levels were found in patients with more advanced disease. Further large-scale studies are needed to validate a possible role for DHEAS as a biochemical marker of tumor stage and to draw robust conclusions on the use of adjuvant chemotherapy.
We would like to thank the Departments of General Surgery and Oncology and all those involved in the care and follow-up of patients and families. We are grateful to Miss Janneke Deurloo for English editing.
Research funding: None declared.
Author contributions: María Celeste Mattone: contributed to data acquisition, analysis, and interpretation and wrote the manuscript with input from all authors. Silvia Gil: contributed to data acquisition, analysis, and interpretation. Mariana Costanzo: contributed to data acquisition, analysis, and interpretation, and contribute to manuscript writing. María Laura Galluzzo Mutti: reviewed, analyzed, and interpreted the histopathology data. Alejandra Casanovas: contributed to the acquisition and analysis of oncology data. Verónica Zaidman: contributed to the acquisition and analysis of laboratory data. Juan Manuel Lazzati: contributed to the acquisition and analysis of laboratory data. Marta Ciaccio: critically reviewed the manuscript and provided feedback. Alicia Belgorosky: critically reviewed the manuscript regarding important intellectual content and provided feedback. Gabriela Guercio: designed the study and critically reviewed the manuscript regarding important intellectual content. Final approval of the version to be published.
Competing interests: The authors declare no conflict of interests.
Informed consent: Informed consent was obtained from all individuals included in this study.
Ethical approval: The local Institutional Review Board deemed the study exempt from review.
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