[Treatment of childhood adrenocortical tumor].
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.
Pereira
Rosana Marques
RM
Unidade de Endocrinologia Pediátrica, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR. sandrini@ufpr.br
Michalkiewicz
Edson
E
Pianovski
Mara Albonei
MA
França
Susana Nesi
SN
Boguszewski
Margaret C S
MC
Cat
Izrail
I
Lacerda Filho
Luiz de
Ld
Sandrini
Romolo
R
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