Histopathological Criteria for Paediatric Adrenocortical Carcinoma

Introduction: Adrenocortical carcinoma (ACC) is diagnosed in paediatric patients at 5 months after symptom onset on average, and 38% die during the first 2.5 years of follow-up. This study aimed to compare the accuracy of Weiss, Van Slooten, and Wieneke histopathological ACC classifications for predicting follow-up prognosis in a paediatric population. Methods: Data were retrieved from medical records of 57 patients aged <18 years who underwent surgical treatment for ACC with surgical follow-up over 6 months or death due to ACC. They were classified into either good (without recurrence/death due to ACC) or poor (with recurrence/death due to ACC) prognosis group. Two expert pathologists classified the ACC surgical specimens according to the Weiss, Van Slooten, and Wieneke criteria. Results: The median follow-up duration was 126 (18–225) months in 38 males (66.7%) and 19 females (33.3%) (median age: 3 [1–6.5] years). The good prognosis group was younger than the poor prognosis group (median age: 3 [1.5–6.2] years vs. 5 [2–10] years). Seventeen (29.8%) patients in the poor prognosis group died due to ACC within the first 50 months of surgical follow-up; the earliest death occurred in the fourth follow-up month, and the majority of deaths occurred within 24 months of follow-up. The accuracies of Weiss, Van Slooten, and Wieneke classification systems were 40%, 47%, and 77%, respectively. Discussion/Conclusion: The Wieneke classification showed the best accuracy but was not sufficiently precise to establish reliable prognosis for ACC in the paediatric population. The Wieneke classification had approximately 95% sensitivity and negative predictive value.

Object Details

• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch

References and Relationships

• Bibliographic citation: Histopathological Criteria for Paediatric Adrenocortical Carcinoma ; volume:95 ; number:4 ; year:2022 ; pages:347-353 ; extent:7 ; Hormone research in paediatrics ; 95, Heft 4 (2022), 347-353 (gesamt 7)

Contributors, Places and Time

• Creator: Paschoalin, Victor P. ; Tucci Júnior, Silvio ; G. Estevanato, Andrey ; B. Tiraboschi, Ricardo ; Antonini, Sonir R. ; Muglia, Valdair F. ; Chahud, Fernando ; Mermejo, Livia M. ; de Bessa Júnior, José ; Fernandes Molina, Carlos A.

Further information

• DOI: 10.1159/000524892
• URN: urn:nbn:de:101:1-2022101300392509029528
• Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 15.08.2025, 7:30 AM CEST