IMPE2023 Free Communications Adrenals and HPA Axis 2 (4 abstracts)
Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-up
Fernanda Bachega 1 , Caio Suartz 1 , Madson Almeida 1 , Vania Brondani 1 , Helaine Charchar 1 , Amanda Lacombe 1 , Sebastião Martins-Filho 1 , Iberê Soares 1 , Maria Claudia Zerbini 1 , Francisco Dénes 2 , Berenice Mendonca 1 , Roberto Lopes 2 , Ana Claudia Latronico 1 & Maria Candida Fragoso 1
1Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. 2Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil
The estimated global prevalence of pediatric adrenocortical tumors (PACTs) is 0.5 cases/million population. However, malignant adrenocortical carcinomas is rare and accounts for 0.2% of all childhood malignancies, with considerable variability in incidence rates per different geographical areas. The south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to identify the epidemiological, clinicopathological and genetic factors associated with the development of metastasis or fatal outcomes in a cohort of patients diagnosed with PACT at a unique tertiary care center in Brazil based on a long-term follow-up study. Additionally, we compiled published data from this same care center to analyze all aforementioned criteria simultaneously, enhancing prognostic factors in the entire cohort. Clinical presentation; tumor hormonal secretion; radiological imaging, histological features (immunohistochemical Ki67% and p53), and genetic data (TP53, MLH1, MSH6, ATRX, ZNRF3, XAF1, BUB1-PINK, IGF-IR) were retrospectively analyzed in 95 patients (71% females). The patients were categorized into three age-groups: group 1: ≤ 3 years of age; group 2: 3 < age ≤ 12 years of age; group 3: 12 < age ≤ 18 years of age. The groups were further subdivided into follow subgroups: metastatic and non-metastatic. Cox's proportional hazards model was used to describe the relationship of variables with time to progression/death, and the results presented as hazard ratios (HR) with confidence intervals. The worst prognosis was associated with those: aged > 3 years (P< 0.05; HR 2.5), high serum levels of 11-desoxicortisol (P< 0.001; HR: 2.9), tumor weight ≥ 200g (P< 0.001; HR: 16.2), tumor size ≥ 5cm (P< 0.05; HR: 5.3), Weiss score ≥ 5 (P< 0.05; HR: 6.2), Wieneke index ≥ 3 (P< 0.001; HR: 25.8) and Ki67 ≥ 15% (P< 0.05; HR: 8.1). Furthermore, patients with MacFarlane stage IV almost had two times shorter overall survival than patients with others stages (P< 0.001). Additionally, the subtractions of BUB1B-PINK1 (< 6.95) expression (P< 0.05) and IGF-IR overexpression (P= 0.0001; HR: 1.84) were associated with malignant behavior. These results helped identify patients who are likely to have an aggressive course; further multicenter prospective studies are required to confirm our results. In conclusion, PACTs with these patterns of prognostic factors could be treated using an adjuvant approach that may improve the overall survival in such patients.
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