title: Rare case of an adrenocortical neoplasm: A case report and review of literature.
abstract: Adrenocortical neoplasms (ACNs) are rare and poorly characterized in infants. The true incidence of ACNs is not well known and it appears to vary substantially across different geographical areas. ACNs are more common in females and two peaks of incidence have been identified: The first year of life and between the age of nine and 16 years. Due to the heterogeneity and rarity of ACNs, their pathological and prognostic classification is challenging. The current study describes the case of a seven-year-old male, who presented to the Department of Pediatric Surgery, University of Siena (Siena, Italy) with a feminization syndrome and increased somatic growth that was associated with a unilateral adrenal mass, which was diagnosed by magnetic resonance imaging. Surgical excision of the mass was performed and histological analysis determined that it was an ACN, with a low risk of malignity; however, the pathological classification of the tumor was challenging. At present, the future behavior of ACNs is unpredictable. Therefore, increasing the knowledge surrounding this type of tumor may aid in its diagnosis, treatment and prognosis. Due to the rarity of pediatric ACNs, no single pediatric oncology center has acquired extensive experience treating this type of tumor. Thus, the initiation of an international tumor registry may aid with the management of patients presenting with ACNs.
authors.affiliation: Department of Pediatric Surgery, University of Siena, Siena 53100, Italy.
authors.forename: Rossella
authors.initials: R
authors.lastname: Angotti
authors.affiliation: Department of Pediatric Surgery, University of Siena, Siena 53100, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Molinaro
authors.affiliation: Department of Pediatric Surgery, University of Siena, Siena 53100, Italy.
authors.forename: Anna Lavinia
authors.initials: AL
authors.lastname: Bulotta
authors.affiliation: Department of Pediatric Surgery, University of Siena, Siena 53100, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: DI Maggio
authors.affiliation: Department of Pediatric Surgery, University of Siena, Siena 53100, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Brandigi
authors.affiliation: Department of Pediatric Surgery, University of Siena, Siena 53100, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Messina
keywords: adrenal glands; infant; neoplasm