Histopathological and Molecular Characteristics of Pediatric Adrenocortical Tumors: where Do we Stand?

Figures

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  Fig. 1. Hematoxylin and eosin (H&E)-stained sections showing morphological features of pediatric adrenal cortical carcinoma. A and B come from a sporadic ACC in a 15-year-old girl and show necrosis and venous invasion respectively. C comes from an ACC in a 5-year-old boy with Li-Fraumeni syndrome and shows profound nuclear pleomorphism and an atypical mitotic figure

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  Fig. 2. Two-step scoring system developed by Picard et al. [24]. In the first step, the COG stage is defined. R0 = complete surgical resection, R1 = microscopic residual after surgical resection, R2 = macroscopic residual after surgical resection (adapted from Picard et al. (2019)). Created in BioRender. De Krijger, R. (2026) https://BioRender.com/kmi3uhs)

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  Fig. 3. Atypia and anaplasia in an ACC from a 5-year-old boy with LFS

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  Fig. 4. Hematoxylin and eosin stainings of adrenocortical tumor from a 7-year-old girl with Beckwith-Wiedemann syndrome. A. Close-up of cellular area featuring numerous mitoses. B. Transition between a cellular tumor area to a less cellular area with marked atypia in cells with ample eosinophilic cytoplasm. C. Transition of epithelioid cellular area (as in A) to spindle cell area. The tumor cells were stained for alpha-inhibin and Melan A; the Ki67 labelling index was 20%

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  Fig. 5. Ki67 immunohistochemistry. a) ACC in a 5-year-old boy with Li-Fraumeni Syndrome (Ki67-LI 45%). b) Example of Ki67 algorithmic automated counting in a whole slide image of an ACC in a 10-year-old girl (Ki67-LI 4.9%). Green signs indicate positive nuclei; red signs indicate negative nuclei

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  Fig. 6. Immunohistochemistry work-up, with possible biomarkers Ki67, Lamin B1, MHC II and IGF-1R, micro-array analysis of micro-RNA, and quantitative real-time PCR of HLA-genes (Created in BioRender. De Krijger, R. (2025) https://BioRender.com/59v5o7h)