Surgical Aspects in the Treatment of Adrenocortical Carcinomas in Children: Data of the GPOH-MET 97 Trial

Hubertus, Jochen; Boxberger, Nicole; Redlich, Antje; Schweinitz, Dietrich von; Vorwerk, Peter

doi:10.1055/s-0032-1304627

Cited by 38 publications

(45 citation statements)

References 10 publications

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“…Consistent with previous studies [3, 9, 15, 16, 26, 34], mixed symptomatology (virilization, Cushing syndrome, and hyperaldosteronism) was the most common presentation of ACC across all age groups in our cohort. One patient presented at 5 months of age with lower extremity weakness and was diagnosed with ectopic extramedullary intradural ACC with 2 intact adrenal glands.…”

Section: Discussionsupporting

confidence: 92%

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

et al. 2018

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Background/Aims: Adrenocortical carcinoma (ACC) is an aggressive childhood cancer. Limited evidence exists on a definite histopathological criterion to differentiate ACC from adrenocortical adenoma. The aim of this study was to investigate the clinicopathological data of children with ACC, identify prognostic factors, and validate a histopathological criterion to differentiate ACC from adrenocortical adenoma. Methods: This retrospective cohort included 41 children, followed at the Mayo Clinic from 1950 to 2017 (onset of symptoms ≤21 years). Outcomes of interest were: alive with no evidence of disease, alive with evidence of disease, and dead of disease. Results: Median age at onset of symptoms was 15.7 years (n = 41; range, 0.2–21 years). Female:male ratio was 3.6: 1. Mixed symptomatology (> 1 hormone abnormality) was the most common presentation (54%, n = 22). Sixty-six percent of patients (n = 27 out of 41) underwent total adrenalectomy. Metastatic disease was more common in children aged > 12 years (p = 0.002 compared to < 4 years). The most common sites of metastases were the liver and lungs. Overall 2-year and 5-year survival rates were 61% (95% CI 45–77) and 46% (95% CI 30–62), respectively. Metastasis at the time of diagnosis was independently associated with poor prognosis (risk ratio 13.7%; 95% CI 3.9–87.7). Weiss criteria (29%) and modified Weiss criteria (33%) were less accurate in younger patients (< 12 years), compared to the Wieneke index (100%). Conclusion: The presence of metastases was an independent prognostic factor. The Wieneke index was the most accurate in predicting clinical outcomes in younger children.

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Section: Discussionsupporting

confidence: 92%

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

et al. 2018

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“…Moreover, in metastasized disease, evidence on treatment options is scarce. Recently published results from the German GPOh-MET 97 trial emphasize the importance of radical surgery in localized disease and suggest that chemotherapy (vincristine/ifosfamide/adriamycin or carboplatin/VP16) in combination with mitotane could yield a survival benefit in metastasized disease (3,11).…”

Section: Adrenocortical Carcinoma In Children: First Population-basedmentioning

confidence: 99%

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

et al. 2014

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Abstract. Adrenocortical carcinoma (ACC) is rare in both adult and pediatric populations. Literature suggests significant differences between children and adults in presentation, histological properties and outcome. The aim of this first nationwide study on pediatric ACC was to describe the incidence, presentation, pathological characteristics, treatment and survival in The Netherlands. All ACC patients aged <20 years at diagnosis and registered in the population-based Netherlands Cancer Registry between 1993 and 2010 were included. Clinical data were extracted from medical records. Archival histological slides were collected via the Dutch Pathology Registry (PALGA). We compared our findings to all clinical studies on pediatric ACC that were found on PubMed. Based on the results, 12 patients were identified: 8 females and 4 males. The median age was 4

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“…O sistema de estadiamento tumoral mais conhecido e empregado na classificação de TAC é a escala de Macfarlane modificada por Sullivan (Tabela 2) (64). Porém, foi observado que este sistema apresentava uma falha na separação prognóstica entre os estágios II e III (65).…”

Section: Estadiamento Tumoralunclassified

“…Therefore, laparotomy is still the most recommended surgical approach [27,63,64]. Despite this, based on the advantages of laparoscopy over open surgery (shorter hospital stay, faster postoperative recovery and better cosmetic results), some services with experience in laparoscopy surgery have reported good results with this technique in pediatric ACTs [65][66][67].…”

Section: Surgerymentioning

confidence: 99%

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A expressão dos marcadores de células-tronco, SF1 e DAX1 no desenvolvimento do córtex adrenal humano e sua associação com a via Wnt/beta-catenina na tumorigênese adrenocortical

Cavalcanti¹

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Autorizo a reprodução e divulgação total ou parcial deste trabalho, por qualquer meio convencional ou eletrônico, para fins de estudo e pesquisa, desde que citada a fonte. Catalogação da PublicaçãoPreparada pela Biblioteca do Serviço de Biblioteca e Documentação Faculdade de Medicina de Ribeirão Preto Universidade de São PauloCavalcanti, Marcelo Machado A expressão dos marcadores de células-tronco, SF1 e DAX1 no desenvolvimento do córtex adrenal humano e sua associação com a via Wnt/ Beta-catenina na tumorigênese adrenocortical, Ribeirão Preto, 2017 Aos grandes amigos que fiz durante esta jornada, que vivenciaram toda esta experiência comigo. À Danila, pela amizade, incentivo, fé e por me ajudar a crescer como pessoa. À Aline, amiga do abraço sincero, pela ternura, paciência, auxílio e incentivo. À Renata e ao Leonardo, por sempre me ajudarem a rir "mesmo que sem querer", ajudando a tornar os dias mais fáceis.À amiga e atual companheira de trabalho, Ana Cláudia, pela parceria, determinação e disposição em ajudar sempre que precisei. Resultados: Em adrenais fetais com 20 e 25 semanas de gestação detectou-se marcação intensa de SF1, DAX1, SOX2 e OCT4 na região subcapsular e marcação leve a moderada e difusa de STAT3 e NANOG. Após as 31 semanas e no período pós-natal, a marcação intensa de SF1 persistiu, porém a marcação de DAX1, STAT3, NANOG e OCT4 diminuiu ou desapareceu. Nos TAC, a marcação nuclear de SF1 foi positiva em 67% das amostras pediátricas e em 19% das amostras de adultos. Ganho de material genômico do NR5A1 foi observado em 71% dos TAC pediátricos e 33% de TAC adultos. Porém observou-se hipoexpressão do RNAm do NR5A1 em 84% dos TAC pediátricos. Nenhum ganho de material genômico ou aumento da expressão do RNAm do NR0B1 foi observado nos TAC pediátricos. Porém em 45% desses tumores detectou-se marcação nuclear. Nos TAC de pacientes adultos, houve aumento da expressão de RNAm de NR0B1 em 89% das amostras. Não se observou marcação de STAT3 nos TAC pediátricos e adultos. Porém nos pacientes adultos, a expressão do RNAm de STAT3 foi significativamente maior nos adenomas do que nos carcinomas. Por sua vez, a marcação nuclear do OCT4 associou-se significativamente com a ocorrência de metástases nos pacientes pediátricos. Nos TAC com a mutação p.S45P no gene da Beta-catenina detectou-se expressão significativamente aumentada do RNAm do NANOG. Além disso, após a inibição in vitro da via Wnt/Beta-catenina nas células tumorais adrenais houve redução significativa na expressão do RNAm de NANOG. Conclusão: Existe um padrão temporal na expressão de SF1, DAX1, STAT3, NANOG e OCT4 durante o desenvolvimento do córtex adrenal fetal, porém apenas a expressão de SF1 permanece no final da gestação e após o nascimento. TAC pediátricos exibem ganho de material gênico, aumento da expressão proteica e aparentemente redução paradoxal do RNAm do SF1 (NR5A1). Marcação nuclear de OCT4 se associa a fenótipo tumoral mais agressivo nos pacientes pediátricos. Parece exisitir interação entre a via Wnt/Beta-catenina e células-tronco, po...

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Surgical Aspects in the Treatment of Adrenocortical Carcinomas in Children: Data of the GPOH-MET 97 Trial

Cited by 38 publications

References 10 publications

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

A expressão dos marcadores de células-tronco, SF1 e DAX1 no desenvolvimento do córtex adrenal humano e sua associação com a via Wnt/beta-catenina na tumorigênese adrenocortical

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