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Journal of Pediatric Biochemistry 2015; 05(04): 109-114
DOI: 10.1055/s-0036-1572321
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Pediatric Adrenocortical Tumors: Clinicopathological Features—An Update

Lucia Salvatorelli
1   Anatomic Pathology Section, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia,” Hospital Policlinico, University of Catania, Catania, Italy
,
Giuseppe Angelico
1   Anatomic Pathology Section, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia,” Hospital Policlinico, University of Catania, Catania, Italy
,
Fabio Motta
1   Anatomic Pathology Section, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia,” Hospital Policlinico, University of Catania, Catania, Italy
,
Andrea Di Cataldo
2   Section of Pediatric Hematology and Oncology Unit, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Italy
,
Pietro Milone
3   Unit of Radiology, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia,” Hospital Policlinico, University of Catania, Catania, Italy
,
Martino Ruggieri
4   Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Gaetano Magro
1   Anatomic Pathology Section, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia,” Hospital Policlinico, University of Catania, Catania, Italy
› Author Affiliations
Further Information

Publication History

23 December 2015

30 December 2015

Publication Date:
03 February 2016 (online)

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Abstract

Pediatric adrenocortical tumors (ACTs) are rare in children. Most tumors are hormonally active with overproduction of androgens and glucocorticoids, and less frequently mineralocorticoids and estrogens. Patients usually present with clinical signs of virilization or Cushing syndrome, while only a minority of ACTs are incidentally discovered. Unfortunately, the clinical behavior of pediatric ACTs is often unpredictable, and the distinction between benign and malignant tumors is still challenging. The present review deals with the main clinical and laboratory features which can help to diagnose pediatric ACTs, with special emphasis on the pathological criteria useful to stratify patients into three different prognostic categories: (1) patients with benign tumors; (2) patients with malignant tumors; and (3) patients with borderline tumors (tumors with indeterminate malignancy). In this regard, general guidelines and histological illustrations are provided to offer a practical approach for a correct identification of morphological predictors of clinical outcome.

Keywords

adrenocortical tumors - pediatric - virilization - Cushing syndrome
 

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