[Childhood adrenocortical tumors].
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.
Pereira
Rosana Marques
RM
Unidade de Endocrinologia, Departamento de Pediatria, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR.
Michalkiewicz
Edson
E
Sandrini
Fabiano
F
Figueiredo
Bonald C
BC
Pianovski
Mara
M
França
Susana Nesi
SN
Boguszewski
Margaret C S
MC
Costa
Orival
O
Cat
Izrail
I
Lacerda Filho
Luiz de
Ld
Sandrini
Romolo
R
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