{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T04:24:35Z","timestamp":1790915075536,"version":"4.1.0"},"reference-count":24,"publisher":"Bioscientifica","issue":"4","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,2,8]]},"abstract":"<jats:p>Histopathological differentiation in pediatric adrenocortical carcinoma (pACC) is difficult and clinical prediction and stratification scores are not evaluated yet. Therefore, this review aims to summarize current evidence on the value and accuracy of the two commonly used scoring systems (Weiss\/Armed Forces Institute of Pathology (AFIP)) pACC. On this base, one might be able to evaluate if patients may benefit from a unique scoring system. For this, we performed a systematic review of the published literature and included 128 patients in our analysis. The majority (72%) of the pACCs had a good clinical course. The follow-up time ranged from 0 to 420 months with a mean age of 5.6 years at diagnosis. Patients with a good clinical course were younger (mean 4.8 years) than patients with a poor outcome (mean 7.6 years). Comparing the two scoring systems, the specificity of the Weiss score was very low (25%), whereas the sensitivity was 100%. According to the AFIP score, specificity (77%) was higher than the Weiss score, whereas the sensitivity of the AFIP score was minimal lower with 92%. Age differences were recognizable as the specificity was lower in infants &lt;4 years (20%) than in older children (32%). In contrast, the specificity of the AFIP score was higher in infants &lt;4 years (82%) than in older age groups (76%). Summarizing our results, we could show that the Weiss score is not a suitable tool for the prediction of malignancy in pACC in comparison with the AFIP score, but further efforts may seek to ensure early and accurate stratification through augmented scoring.<\/jats:p>","DOI":"10.1530\/erc-22-0259","type":"journal-article","created":{"date-parts":[[2023,2,8]],"date-time":"2023-02-08T11:48:54Z","timestamp":1675856934000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":15,"title":["Prognostic value of the Weiss and Wieneke (AFIP) scoring systems in pediatric ACC \u2013 a mini review"],"prefix":"10.1530","volume":"30","author":[{"given":"Maria","family":"Riedmeier","sequence":"first","affiliation":[{"name":"University Children\u2019s Hospital, Department of Pediatric Hematology, Oncology and Stem cell transplantation, University of Wuerzburg, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lester D R","family":"Thompson","sequence":"additional","affiliation":[{"name":"Head and Neck Pathology Consultations, Los Angeles, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos Augusto Fernandes","family":"Molina","sequence":"additional","affiliation":[{"name":"Department of Surgery and Anatomy, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Boris","family":"Decarolis","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology and Hematology, University Children\u2019s Hospital of Cologne, Medical Faculty, Cologne, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"H\u00e4rtel","sequence":"additional","affiliation":[{"name":"University Children\u2019s Hospital, Department of Pediatric Hematology, Oncology and Stem cell transplantation, University of Wuerzburg, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul-G","family":"Schlegel","sequence":"additional","affiliation":[{"name":"University Children\u2019s Hospital, Department of Pediatric Hematology, Oncology and Stem cell transplantation, University of Wuerzburg, Wuerzburg, Germany."},{"name":"Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre, Wuerzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Fassnacht","sequence":"additional","affiliation":[{"name":"Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre, Wuerzburg, Germany"},{"name":"Department of Medicine, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, Wuerzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2802-4034","authenticated-orcid":true,"given":"Verena","family":"Wiegering","sequence":"additional","affiliation":[{"name":"University Children\u2019s Hospital, Department of Pediatric Hematology, Oncology and Stem cell transplantation, University of Wuerzburg, Wuerzburg, Germany."},{"name":"Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre, Wuerzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"New England Journal of Medicine","first-page":"2243","article-title":"Chromosome 1p and 11q deletions and outcome in neuroblastoma","volume":"353","author":"Attiyeh","year":"2005","unstructured":"Attiyeh EFLondon WBMoss\u00e9 YPWang QWinter CKhazi DMcgrady PWSeeger RCLook ATShimada H2005Chromosome 1p and 11q deletions and outcome in neuroblastoma. 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(https:\/\/doi.org\/10.1016\/j.mce.2011.10.015)"},{"key":"bib18","series-title":"Journal of Clinical Oncology","first-page":"67","article-title":"Adrenocortical carcinoma in children: a study of 40 cases","volume":"8","author":"Ribeiro","year":"1990","unstructured":"Ribeiro RCSandrini Neto RSSchell MJLacerda LSambaio GACat I1990Adrenocortical carcinoma in children: a study of 40 cases. Journal of Clinical Oncology867\u201374. (https:\/\/doi.org\/10.1200\/JCO.1990.8.1.67)"},{"key":"bib19","series-title":"Cancers (Basel)","article-title":"Adrenocortical carcinoma in childhood: a systematic review","volume":"13","author":"Riedmeier","year":"2021","unstructured":"Riedmeier MDecarolis BHaubitz IM\u00fcller SUttinger KB\u00f6rner KReibetanz JWiegering AH\u00e4rtel CSchlegel PG2021Adrenocortical carcinoma in childhood: a systematic review. Cancers (Basel)135266. 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