title: [Childhood adrenocortical tumors].
abstract: Adrenocortical tumors (ACT) in children are uncommon. However, the incidence of these tumors in Paraná, Brazil, is 15 times higher than that worldwide. We describe the clinical, laboratory and treatment characteristics and outcome of 125 patients treated in a single institution in the State of Paraná. The median age at diagnosis was 4.3 years, with a female:male ratio of 2.6:1. The most common forms of presentation were isolated virilization (51.2%) and virilization and Cushing's syndrome (42%). Nonfunctioning tumors comprised 4.8% of the cases. Two patients (1.6%) had isolated Cushing's syndrome and 1 (0.8%) had Conn's syndrome. Fifty-six percent presented hypertension. Surgery is the only curative treatment. Our data show that disease stage 1, absence of spillage during surgery and absence of intravenous thrombus were associated with better survival rates.
authors.affiliation: Unidade de Endocrinologia, Departamento de Pediatria, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR.
authors.forename: Rosana Marques
authors.initials: RM
authors.lastname: Pereira
authors.forename: Edson
authors.initials: E
authors.lastname: Michalkiewicz
authors.forename: Fabiano
authors.initials: F
authors.lastname: Sandrini
authors.forename: Bonald C
authors.initials: BC
authors.lastname: Figueiredo
authors.forename: Mara
authors.initials: M
authors.lastname: Pianovski
authors.forename: Susana Nesi
authors.initials: SN
authors.lastname: França
authors.forename: Margaret C S
authors.initials: MC
authors.lastname: Boguszewski
authors.forename: Orival
authors.initials: O
authors.lastname: Costa
authors.forename: Izrail
authors.initials: I
authors.lastname: Cat
authors.forename: Luiz de
authors.initials: Ld
authors.lastname: Lacerda Filho
authors.forename: Romolo
authors.initials: R
authors.lastname: Sandrini
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000628:therapy; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis