Pediatric adrenocortical tumors cohort characteristics and long-term follow-up at a single Argentinian tertiary center
Author:
Mattone María Celeste12, Gil Silvia1, Costanzo Mariana1, Galluzzo Mutti María Laura1ORCID, Casanovas Alejandra13, Zaidman Verónica1, Lazzati Juan Manuel1, Ciaccio Marta1, Belgorosky Alicia12, Guercio Gabriela12ORCID
Affiliation:
1. Hospital de Pediatría Garrahan , Buenos Aires , Argentina 2. Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET) , Buenos Aires , Argentina 3. Instituto Nacional del Cáncer , Buenos Aires , Argentina
Abstract
Abstract
Pediatric adrenocortical tumors are rare and heterogeneous endocrine malignancies.
Objectives
To report clinical, biochemical, and histological features, staging, and therapeutic interventions in a cohort of 28 patients treated at a single tertiary center.
Methods
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.
Results
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.
Conclusions
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.
Publisher
Walter de Gruyter GmbH
Subject
Endocrinology,Endocrinology, Diabetes and Metabolism,Pediatrics, Perinatology and Child Health
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