Adrenocortical carcinoma in childhood: a systematic review

Please always quote using this URN: urn:nbn:de:bvb:20-opus-248507
  • 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 mutationAdrenocortical 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% (n = 65) — distant metastases at relapse. Patients < 4 years old had a different distribution of tumor stages and hormone activity and better overall survival (p < 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.show moreshow less

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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
URN:urn:nbn:de:bvb:20-opus-248507
Document Type:Journal article
Faculties:Medizinische Fakultät / Klinik und Poliklinik für Allgemein-, Viszeral-, Gefäß- und Kinderchirurgie (Chirurgische Klinik I)
Medizinische Fakultät / Kinderklinik und Poliklinik
Medizinische Fakultät / Theodor-Boveri-Institut für Biowissenschaften
Medizinische Fakultät / Medizinische Klinik und Poliklinik I
Medizinische Fakultät / Comprehensive Cancer Center Mainfranken
Language:English
Parent Title (English):Cancers
ISSN:2072-6694
Year of Completion:2021
Volume:13
Issue:21
Article Number:5266
Source:Cancers (2021) 13:21, 5266. https://doi.org/10.3390/cancers13215266
DOI:https://doi.org/10.3390/cancers13215266
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Tag:pediatric adrenocortical adenoma; pediatric adrenocortical cancer; pediatric adrenocortical tumor; prognostic factors; therapy
Release Date:2023/05/26
Date of first Publication:2021/10/20
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung 4.0 International