Pediatric adrenocortical tumors cohort characteristics and long-term follow-up at a single Argentinian tertiary center

Mattone, Maria Celeste; Gil, Silvia; Costanzo, Mariana; Galluzzo, Laura; Casanovas, Alejandra; Zaidman, Verónica; Lazzati, Juan Manuel; Ciaccio, Marta; Belgorosky, Alicia; Guercio, Gabriela

doi:10.1515/jpem-2021-0392

Cited by 4 publications

(1 citation statement)

References 31 publications

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“…Pediatric malignant patients have benefited from the rapid progress in tumor treatment as the optimization of multimodality therapy and early tumor screening has dramatically improved the survival rate of pediatric malignant tumors (9)(10)(11)(12)(13). Given the low incidence of pediatric adrenal tumors, the small sample size, and the short follow-up time of prior studies on pediatric adrenal malignancies, the clinicopathological characteristics, treatment status, and prognosis are not well understood (2,4,(11)(12)(13)(14)(15). Here, we used the Surveillance, Epidemiology, and End Results (SEER) database to study pediatric malignancies of adrenal glands to explore the long-term follow-up survival outcomes and prognostic risk analysis of pediatric adrenal malignancies.…”

Section: Introductionmentioning

confidence: 99%

Long-term survival outcomes of pediatric adrenal malignancies: An analysis with the upstaged SEER registry during 2000-2019

Luo

et al. 2022

Front. Endocrinol.

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ObjectiveTo investigate the clinicopathological characteristics and long-term survival outcomes of pediatric adrenal malignancies.MethodThis study retrospectively analyzed children with pathologically confirmed pediatric adrenal malignancies from Surveillance, Epidemiology, and End Results Database from 2000 to 2019. Kaplan-Meier curve was used to assess the overall survival (OS) and cancer-special survival (CSS), and the Log-Rank method was used to calculate statistical differences. Cox proportional hazards model and Fine-and-Grey model were used to calculate the hazard ratio (HR) of all-cause mortality risk and the sub-distribution HR (sHR) of disease-specific mortality risk, respectively, and their corresponding 95% confidence intervals (CI).Results1601 children were included in the study in which 1335 (83.4%) neuroblastoma, 151 (9.4%) ganglioneuroblastoma, 89 (5.6%) adrenocortical carcinoma, and 26 (1.6%) were diagnosed with other types malignancies. Metastatic disease accounted for the largest proportion (69.3%), and the proportion of metastases diagnosed by neuroblastoma was higher than that of adrenocortical carcinoma and ganglioneuroblastoma (73.9% vs. 45.7% vs. 47.2%). The 5-year OS and CSS of all cohort were 69.5% and 70.5%, respectively. Adrenal cortical carcinoma had the worst prognosis, with 5-year OS and CSS of 52.5% and 53.1%, respectively. Patients in recent years had no better OS and CSS than in previous years at diagnosis. The tumor stage remained the main prognostic predictor. Compared to metastatic adrenal tumors, the risk of all-cause mortality (adjusted HR: 0.12, 95% CI: 0.06-0.25, P < 0.001) and the risk of disease-specific mortality (adjusted sHR: 0.11, 95% CI: 0.05-0.25, P<0.001) was significantly lower for patients with localized diseases. Additionally, higher age, adrenal cortical carcinoma, and lack of complete tumor resection are independent risk factors for poor prognosis. Furthermore, it was found that the prognosis of patients who received chemotherapy was worse than those who did not, mainly because the former mostly had metastasis at the presentation and complete resection of the tumor cannot be achieved.ConclusionThe clinicopathological characteristics of pediatric adrenal malignancies have not changed significantly in the past two decades, while the prognosis of patients has improved. Early diagnosis of disease and complete resection of local tumors are the keys to improving prognosis.

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Section: Introductionmentioning

confidence: 99%

Long-term survival outcomes of pediatric adrenal malignancies: An analysis with the upstaged SEER registry during 2000-2019

Luo

et al. 2022

Front. Endocrinol.

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International consensus on mitotane treatment in pediatric patients with adrenal cortical tumors: indications, therapy, and management of adverse effects

Riedmeier,

Antonini,

Brandalise

et al. 2024

European Journal of Endocrinology

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Objective Mitotane is an important cornerstone in the treatment of pediatric adrenal cortical tumors (pACC), but experience with the drug in the pediatric age group is still limited and current practice is not guided by robust evidence. Therefore, we have compiled international consensus statements from pACC experts on mitotane indications, therapy, and management of adverse effects. Methods A Delphi method with three rounds of questionnaires within the pACC expert consortium of the international network groups ENSAT-PACT and ICPACT was used to create 21 final consensus statements. Results We divided the statements into 4 groups: environment, indications, therapy, and adverse effects. We reached a clear consensus for mitotane treatment for advanced pACC with stage III and IV and with incomplete resection/tumor spillage. For stage II patients mitotane is not generally indicated.The timing of initiating mitotane therapy depends on the clinical condition of the patient and the setting of the planned therapy. We recommend a starting dose of 50 mg/kg/d (1500 mg/m²/d) which can be increased up to 4000 mg/m2/d. Blood levels should range between 14-20 mg/L Duration of mitotane treatment depends on the clinical risk profile and tolerability. Mitotane treatment causes adrenal insufficiency in virtually all patients requiring glucocorticoid replacement shortly after beginning. As the spectrum of adverse effects of mitotane is wide-ranging and can be life-threatening, frequent clinical and neurological examinations (every 2 to 4 weeks), along with evaluation and assessment of laboratory values are required. Conclusion Delphi method enabled us to propose an expert consensus statement, which may guide clinicians, further adapted by local norms and the individual patient setting. In order to generate evidence, well-constructed studies should be the focus of future efforts.

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Assessment of prognostic factors in pediatric adrenocortical tumors: the modified pediatric S-GRAS score in an international multicenter cohort—a work from the ENSAT-PACT working group

Riedmeier,

Agarwal,

Antonini

et al. 2024

European Journal of Endocrinology

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Objective Pediatric adrenocortical carcinoma (pACC) is rare, and prognostic stratification remains challenging. We aimed to confirm the prognostic value of the previously published pediatric scoring system (pS-GRAS) in an international multicenter cohort. Design Analysis of pS-GRAS items of pACC from six countries in collaboration of ENSAT-PACT, GPOH-MET and IC-PACT. Methods We received patient data of the pS-GRAS items including survival information from nine centers. PS-GRAS score was calculated as a sum of tumor stage (1 = 0; 2–3 = 1; 4 = 2 points), grade (Ki67 index: 0–9%=0; 10–19%=1; ≥ 20%=2 points), resection status (R0 = 0; RX/R1/R2 = 1 point), age (<4 years = 0; ≥ 4 years = 1 point), and hormone production (androgen production = 0; glucocorticoid-/mixed/-no hormone production = 1 point) generating eight scores and four groups (1: 0–2, 2: 3–4, 3: 5, 4: 6–7). Primary endpoint was overall survival (OS). Results We included 268 patients with median age of 4 years. The analysis of the pS-GRAS score showed a significantly favorable prognosis in patients with a lower scoring compared to higher scoring groups (5-year OS: group 1 98%; group 2 87%(HR of death 3.6, 95% CI of HR 1.6-8.2); group 3 43%(HR of death 2.8, 95% CI 1.9–4.4); group 4: OS 18%(HR of death 2.1, 95% CI 1.7-2.7)). In the multivariable analysis age (HR of death 3.5, 95% CI 1.8-7.0), resection status (HR of death 5.5, 95% CI 2.7-11.1), tumor stage (HR of death 1.9, 95%-CI of HR 1.2-3.0) and Ki67-index (HR of death 1.7, 95% CI 1.2-2.4) remained strong independent outcome predictors. Especially infants < 4 years showed more often low risk constellations with a better OS for all tumor stages. Conclusion In an international multicenter study, we confirmed that the pS-GRAS score is strongly associated with overall survival among patients with pACC. Age, resection status, stage and Ki67-index are important parameters for risk stratification.

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Pediatric adrenocortical tumors cohort characteristics and long-term follow-up at a single Argentinian tertiary center

Cited by 4 publications

References 31 publications

Long-term survival outcomes of pediatric adrenal malignancies: An analysis with the upstaged SEER registry during 2000-2019

Long-term survival outcomes of pediatric adrenal malignancies: An analysis with the upstaged SEER registry during 2000-2019

International consensus on mitotane treatment in pediatric patients with adrenal cortical tumors: indications, therapy, and management of adverse effects

Assessment of prognostic factors in pediatric adrenocortical tumors: the modified pediatric S-GRAS score in an international multicenter cohort—a work from the ENSAT-PACT working group

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