title: [Treatment of childhood adrenocortical tumor].
abstract: Adrenocortical tumors (ACT) in children are uncommon. However, the incidence of these tumors in Paraná is 15 times higher than that worldwide. A germline mutation, R337H TP53, present in more than 95% of our patients, is probably the reason for the higher incidence in our state. A hundred twenty-five patients were treated in the period of 1966 to 2003. Surgery is the only curative treatment. In our experience, disease stage I, absence of spillage during surgery and absence of intravenous thrombus are associated with better survival rates. Preliminary data with the combination of etoposide, doxorubicin, cisplatin, and mitotane have shown that in some patients a complete remission is observed both of the tumor and metastasis. Side effects due to these drugs are common and adrenal insufficiency may occur. Glucocorticoid and mineralocorticoid reposition should be done with 2 to 3 times the physiological doses.
authors.affiliation: Unidade de Endocrinologia Pediátrica, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR. sandrini@ufpr.br
authors.forename: Rosana Marques
authors.initials: RM
authors.lastname: Pereira
authors.forename: Edson
authors.initials: E
authors.lastname: Michalkiewicz
authors.forename: Mara Albonei
authors.initials: MA
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: 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: D000306:Adrenal Cortex Neoplasms / Q000188:drug therapy / Q000601:surgery; D018268:Adrenocortical Carcinoma / Q000188:drug therapy / Q000601:surgery; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D016019:Survival Analysis