Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases
Background: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents wi...
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MDPI AG
2023-08-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/15/17/4296 |
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| author | Michaela Kuhlen Pascal Mier Marina Kunstreich Lienhard Lessel Christoph Slavetinsky Jörg Fuchs Guido Seitz Paul-Martin Holterhus Stefan A. Wudy Christian Vokuhl Michael C. Frühwald Peter Vorwerk Antje Redlich |
| author_facet | Michaela Kuhlen Pascal Mier Marina Kunstreich Lienhard Lessel Christoph Slavetinsky Jörg Fuchs Guido Seitz Paul-Martin Holterhus Stefan A. Wudy Christian Vokuhl Michael C. Frühwald Peter Vorwerk Antje Redlich |
| author_sort | Michaela Kuhlen |
| collection | DOAJ |
| description | Background: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents with COG stage II and III ACC registered in the MET studies were included. The five-item and pS-GRAS score were retrospectively calculated. Results: By December 2021, 55 patients with stage II and III (stage II <i>n</i> = 18, stage III <i>n</i> = 37) had been reported. Median age was 4.3 years [0.1–17.8], median duration of follow-up 6.0 years [0–16.7]. 3-year event-free survival (EFS) rate was 76.5% and 49.8% (<i>p</i> = 0.088), respectively. In stage II tumors, neither the five-item score (<i>p</i> = 0.872) nor pS-GRAS grouping (<i>p</i> = 0.218) had any effect as prognostic factors. In stage III patients, EFS was impaired in tumors with unfavorable histology according to the five-item score (100% vs. 30.8%, <i>p</i> = 0.018). No difference was observed for pS-GRAS groups (<i>p</i> = 0.798). Conclusions: In patients with COG stage III, but not stage II, the five-item score affected EFS. Further studies are needed to identify patients at risk in COG stage II. |
| first_indexed | 2024-03-10T23:27:00Z |
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| id | doaj.art-bc4fb899e0ca4f959db57ea9c052d085 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-10T23:27:00Z |
| publishDate | 2023-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-bc4fb899e0ca4f959db57ea9c052d0852023-11-19T07:55:49ZengMDPI AGCancers2072-66942023-08-011517429610.3390/cancers15174296Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging CasesMichaela Kuhlen0Pascal Mier1Marina Kunstreich2Lienhard Lessel3Christoph Slavetinsky4Jörg Fuchs5Guido Seitz6Paul-Martin Holterhus7Stefan A. Wudy8Christian Vokuhl9Michael C. Frühwald10Peter Vorwerk11Antje Redlich12Pediatrics and Adolescents Medicine, Faculty of Medicine, University of Augsburg, 86156 Augsburg, GermanyPediatric Hematology/Oncology, Department of Pediatrics, Otto von Guericke University Children’s Hospital, 39106 Magdeburg, GermanyPediatrics and Adolescents Medicine, Faculty of Medicine, University of Augsburg, 86156 Augsburg, GermanyPediatric Hematology/Oncology, Department of Pediatrics, Otto von Guericke University Children’s Hospital, 39106 Magdeburg, GermanyDepartment of Paediatric Surgery and Paediatric Urology, University Children’s Hospital Tuebingen, 72076 Tuebingen, GermanyDepartment of Paediatric Surgery and Paediatric Urology, University Children’s Hospital Tuebingen, 72076 Tuebingen, GermanyDepartment of Pediatric Surgery and Urology, University Hospital Giessen-Marburg, 35043 Marburg, GermanyDivision of Paediatric Endocrinology and Diabetes, Department of Paediatrics, University Hospital Schleswig-Holstein, Christian-Albrechts-University Kiel, 24105 Kiel, GermanyPaediatric Endocrinology & Diabetology, Steroid Research & Mass Spectrometry Unit, Centre of Child and Adolescent Medicine, Justus Liebig University, 35392 Giessen, GermanySection of Pediatric Pathology, Institute of Pathology, University of Bonn, 53127 Bonn, GermanyPediatrics and Adolescents Medicine, Faculty of Medicine, University of Augsburg, 86156 Augsburg, GermanyPediatric Hematology/Oncology, Department of Pediatrics, Otto von Guericke University Children’s Hospital, 39106 Magdeburg, GermanyPediatric Hematology/Oncology, Department of Pediatrics, Otto von Guericke University Children’s Hospital, 39106 Magdeburg, GermanyBackground: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents with COG stage II and III ACC registered in the MET studies were included. The five-item and pS-GRAS score were retrospectively calculated. Results: By December 2021, 55 patients with stage II and III (stage II <i>n</i> = 18, stage III <i>n</i> = 37) had been reported. Median age was 4.3 years [0.1–17.8], median duration of follow-up 6.0 years [0–16.7]. 3-year event-free survival (EFS) rate was 76.5% and 49.8% (<i>p</i> = 0.088), respectively. In stage II tumors, neither the five-item score (<i>p</i> = 0.872) nor pS-GRAS grouping (<i>p</i> = 0.218) had any effect as prognostic factors. In stage III patients, EFS was impaired in tumors with unfavorable histology according to the five-item score (100% vs. 30.8%, <i>p</i> = 0.018). No difference was observed for pS-GRAS groups (<i>p</i> = 0.798). Conclusions: In patients with COG stage III, but not stage II, the five-item score affected EFS. Further studies are needed to identify patients at risk in COG stage II.https://www.mdpi.com/2072-6694/15/17/4296adrenocortical carcinomachildren and adolescentslocally advanced disease |
| spellingShingle | Michaela Kuhlen Pascal Mier Marina Kunstreich Lienhard Lessel Christoph Slavetinsky Jörg Fuchs Guido Seitz Paul-Martin Holterhus Stefan A. Wudy Christian Vokuhl Michael C. Frühwald Peter Vorwerk Antje Redlich Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases Cancers adrenocortical carcinoma children and adolescents locally advanced disease |
| title | Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases |
| title_full | Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases |
| title_fullStr | Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases |
| title_full_unstemmed | Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases |
| title_short | Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases |
| title_sort | locally advanced adrenocortical carcinoma in children and adolescents enigmatic and challenging cases |
| topic | adrenocortical carcinoma children and adolescents locally advanced disease |
| url | https://www.mdpi.com/2072-6694/15/17/4296 |
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