[Treatment of childhood adrenocortical tumor].

Pereira, Rosana Marques; Michalkiewicz, Edson; Pianovski, Mara Albonei; França, Susana Nesi; Boguszewski, Margaret C S; Cat, Izrail; Lacerda Filho, Luiz de; Sandrini, Romolo

[Treatment of childhood adrenocortical tumor]. / Tratamento do tumor do córtex adrenal na infância.

Pereira, Rosana Marques; Michalkiewicz, Edson; Pianovski, Mara Albonei; França, Susana Nesi; Boguszewski, Margaret C S; Cat, Izrail; Lacerda Filho, Luiz de; Sandrini, Romolo.

Afiliação

Pereira RM; Unidade de Endocrinologia Pediátrica, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR. sandrini@ufpr.br

Arq Bras Endocrinol Metabol ; 49(5): 747-52, 2005 Oct.

Article em Pt | MEDLINE | ID: mdl-16444357

RESUMO

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.

Assuntos

Neoplasias do Córtex Suprarrenal; Carcinoma Adrenocortical; Neoplasias do Córtex Suprarrenal/tratamento farmacológico; Neoplasias do Córtex Suprarrenal/cirurgia; Carcinoma Adrenocortical/tratamento farmacológico; Carcinoma Adrenocortical/cirurgia; Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico; Criança; Pré-Escolar; Terapia Combinada; Feminino; Humanos; Lactente; Masculino; Estadiamento de Neoplasias; Prognóstico; Análise de Sobrevida

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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias do Córtex Suprarrenal / Carcinoma Adrenocortical Tipo de estudo: Prognostic_studies Limite: Child / Child, preschool / Female / Humans / Infant / Male Idioma: Pt Revista: Arq Bras Endocrinol Metabol Ano de publicação: 2005 Tipo de documento: Article País de publicação: Brasil

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