title: Adrenal cortical carcinoma in a child, a rare cause of paediatric endocrinopathy: case report and literature review.
abstract: Adrenal cortical carcinomas (ACC) are rare tumours, most commonly reported in adult patients. However, an important peak in incidence occurs in paediatric patients. ACC is a rare cause of paediatric endocrinopathy which may masquerade as a non-neoplastic disease process. Herein we present ACC in a five-year-old female patient. Histopathological features associated with poor outcome included tumour weight >500 g, tumour size >10.5 cm, invasive properties, confluent tumour necrosis, high nuclear grade and high proliferation index assessed by Ki67 immunohistochemistry. This article focuses on clinical features, treatment, pathological characteristics, evolving classification and genetic significance of ACC in paediatric patients.
authors.affiliation: Anatomical Pathology, National Health Laboratory Service, Faculty of Health Sciences, University of the Witwatersrand, Gauteng, South Africa. sugeshnee.pather@nhls.ac.za
authors.forename: Sugeshnee
authors.initials: S
authors.lastname: Pather
authors.forename: Biance
authors.initials: B
authors.lastname: Rowe
authors.forename: Dawn van der
authors.initials: Dv
authors.lastname: Byl
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000150:complications* / Q000378:metabolism / Q000473:pathology*; D018268:Adrenocortical Carcinoma / Q000150:complications* / Q000378:metabolism / Q000473:pathology*; D002675:Child, Preschool; D004700:Endocrine System Diseases / Q000209:etiology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008940:Mitotic Index