Adrenocortical Carcinoma in Childhood: A Systematic Review

Adrenocortical tumors are rare in children. This systematic review summarizes the published evidence on pediatric adrenocortical carcinoma (ACC) to provide a basis for a better understanding of the disease, investigate new molecular biomarkers and therapeutic targets, and define which patients may b...

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Autores principales:	Maria Riedmeier, Boris Decarolis, Imme Haubitz, Sophie Müller, Konstantin Uttinger, Kevin Börner, Joachim Reibetanz, Armin Wiegering, Christoph Härtel, Paul-Gerhardt Schlegel, Martin Fassnacht, Verena Wiegering
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	pediatric adrenocortical cancer pediatric adrenocortical adenoma pediatric adrenocortical tumor prognostic factors therapy Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/a3a480832e5b4c6da1e1386a72360f16
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spelling	oai:doaj.org-article:a3a480832e5b4c6da1e1386a72360f162021-11-11T15:26:42ZAdrenocortical Carcinoma in Childhood: A Systematic Review10.3390/cancers132152662072-6694https://doaj.org/article/a3a480832e5b4c6da1e1386a72360f162021-10-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/21/5266https://doaj.org/toc/2072-6694Adrenocortical tumors are rare in children. This systematic review summarizes the published evidence on pediatric adrenocortical carcinoma (ACC) to provide a basis for a better understanding of the disease, investigate new molecular biomarkers and therapeutic targets, and define which patients may benefit from a more aggressive therapeutic approach. We included 137 studies with 3680 ACC patients (~65% female) in our analysis. We found no randomized controlled trials, so this review mainly reflects retrospective data. Due to a specific mutation in the TP53 gene in ~80% of Brazilian patients, that cohort was analyzed separately from series from other countries. Hormone analysis was described in 2569 of the 2874 patients (89%). Most patients were diagnosed with localized disease, whereas 23% had metastasis at primary diagnosis. Only 72% of the patients achieved complete resection. In 334 children (23%), recurrent disease was reported: 81%—local recurrence, 19% (<i>n</i> = 65)—distant metastases at relapse. Patients < 4 years old had a different distribution of tumor stages and hormone activity and better overall survival (<i>p</i> < 0.001). Although therapeutic approaches are typically multimodal, no consensus is available on effective standard treatments for advanced ACC. Thus, knowledge regarding pediatric ACC is still scarce and international prospective studies are needed to implement standardized clinical stratifications and risk-adapted therapeutic strategies.Maria RiedmeierBoris DecarolisImme HaubitzSophie MüllerKonstantin UttingerKevin BörnerJoachim ReibetanzArmin WiegeringChristoph HärtelPaul-Gerhardt SchlegelMartin FassnachtVerena WiegeringMDPI AGarticlepediatric adrenocortical cancerpediatric adrenocortical adenomapediatric adrenocortical tumorprognostic factorstherapyNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5266, p 5266 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	pediatric adrenocortical cancer pediatric adrenocortical adenoma pediatric adrenocortical tumor prognostic factors therapy Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	pediatric adrenocortical cancer pediatric adrenocortical adenoma pediatric adrenocortical tumor prognostic factors therapy Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Maria Riedmeier Boris Decarolis Imme Haubitz Sophie Müller Konstantin Uttinger Kevin Börner Joachim Reibetanz Armin Wiegering Christoph Härtel Paul-Gerhardt Schlegel Martin Fassnacht Verena Wiegering Adrenocortical Carcinoma in Childhood: A Systematic Review
description	Adrenocortical tumors are rare in children. This systematic review summarizes the published evidence on pediatric adrenocortical carcinoma (ACC) to provide a basis for a better understanding of the disease, investigate new molecular biomarkers and therapeutic targets, and define which patients may benefit from a more aggressive therapeutic approach. We included 137 studies with 3680 ACC patients (~65% female) in our analysis. We found no randomized controlled trials, so this review mainly reflects retrospective data. Due to a specific mutation in the TP53 gene in ~80% of Brazilian patients, that cohort was analyzed separately from series from other countries. Hormone analysis was described in 2569 of the 2874 patients (89%). Most patients were diagnosed with localized disease, whereas 23% had metastasis at primary diagnosis. Only 72% of the patients achieved complete resection. In 334 children (23%), recurrent disease was reported: 81%—local recurrence, 19% (<i>n</i> = 65)—distant metastases at relapse. Patients < 4 years old had a different distribution of tumor stages and hormone activity and better overall survival (<i>p</i> < 0.001). Although therapeutic approaches are typically multimodal, no consensus is available on effective standard treatments for advanced ACC. Thus, knowledge regarding pediatric ACC is still scarce and international prospective studies are needed to implement standardized clinical stratifications and risk-adapted therapeutic strategies.
format	article
author	Maria Riedmeier Boris Decarolis Imme Haubitz Sophie Müller Konstantin Uttinger Kevin Börner Joachim Reibetanz Armin Wiegering Christoph Härtel Paul-Gerhardt Schlegel Martin Fassnacht Verena Wiegering
author_facet	Maria Riedmeier Boris Decarolis Imme Haubitz Sophie Müller Konstantin Uttinger Kevin Börner Joachim Reibetanz Armin Wiegering Christoph Härtel Paul-Gerhardt Schlegel Martin Fassnacht Verena Wiegering
author_sort	Maria Riedmeier
title	Adrenocortical Carcinoma in Childhood: A Systematic Review
title_short	Adrenocortical Carcinoma in Childhood: A Systematic Review
title_full	Adrenocortical Carcinoma in Childhood: A Systematic Review
title_fullStr	Adrenocortical Carcinoma in Childhood: A Systematic Review
title_full_unstemmed	Adrenocortical Carcinoma in Childhood: A Systematic Review
title_sort	adrenocortical carcinoma in childhood: a systematic review
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/a3a480832e5b4c6da1e1386a72360f16
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Adrenocortical Carcinoma in Childhood: A Systematic Review

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