OBJECTIVE: To evaluate treatment outcomes in Wilms' tumor (WT). MATERIALS AND METHODS: We studied 53 children with median age of 2 years with WT, stages I-19, II-14, III-12, IV-6 and V-2. Treatment consisted of surgical excision plus adjuvant (40 children) or neoadjuvant and adjuvant chemotherapy (unresectable tumor, n = 8, or caval tumor extension, n = 5). Chemotherapy and radiotherapy followed protocols of Brazilian Wilms' Tumor Study Group excepting 16 cases with stage I disease that received a short duration postoperative treatment with vincristine (VCR - 11 doses) and dactinomycin (AMD - 4 doses). Relapsed WT was treated with multiagent regimens including cisplatin/carboplatin, cyclophosphamide, ifosfamide and etoposide. One patient with resistant relapsed WT was treated by high-dose conditioning chemotherapy with stem cell rescue. RESULTS: Overall and disease-free survival rates at 5 years were respectively 88.2 ± 5.0% and 76.7 ± 6.6%. Short duration therapy for stage I tumor showed a disease-free survival rate of 100% in a median time of 101 months (range 14 to 248 months). Overall and disease-free survival of 10 patients with recurrent WT at 5 years was 42.8%. The child treated with high-dose chemotherapy plus stem cell transplant is alive without evidence of disease 84 months from relapse. CONCLUSION: The postoperative chemotherapy in stage I disease can be reduced without compromising the cure rate. The treatment of unfavorable stage III and IV disease or relapsed tumor remains a challenge.

OBJETIVO: Relatar a experiência no uso do OK-432 para tratamento de linfangiomas em crianças. MÉTODOS: Estudo retrospectivo de 19 crianças com linfangioma tratadas com OK-432 no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto - USP, durante o período de 1999 a 2003. RESULTADOS: Todos os pacientes apresentaram alguma resposta ao OK-432, 12 pacientes apresentaram regressão total, sete apresentaram regressão parcial variando de 50% a 80%. Os pacientes apresentaram febre após a aplicação da droga com duração de 2 a 10 dias. Não se observaram cicatrizes após a aplicação do OK-432. CONCLUSÕES: A droga OK-432 é segura, eficaz e pode ser utilizada como primeira escolha no tratamento de pacientes com linfangiomas devido à excelente resposta, podendo tornar desnecessária a realização de cirurgia. Em pacientes com resposta parcial, podem ser realizadas novas aplicações de OK-432 ou cirurgia menos mutilante, devido à redução das dimensões da lesão.

OBJECTIVE: To report the experience with OK-432 therapy for lymphangioma in children. METHODS: Retrospective study of 19 children with lymphangioma treated with OK-432 in Ribeirão Preto, state of São Paulo, Brazil, between 1999 and 2003. RESULTS: All patients presented response to OK-432, 12 had total shrinkage and seven had partial shrinkage varying from 50% to 80%. Patients had fever after injections of OK-432 for 2 to 10 days, no damage to the overlying skin was observed. CONCLUSION: OK-432 is safe, effective and can be used as primary choice of treatment of patients with lymphangiomas because of the excellent response. In these cases surgery should not be necessary. In patients with partial regression new injections of OK-432 must be used to shrink the lesion. Thereby safely surgery could be made.

O tratamento da Síndrome Mielodisplásica (SMD) na criança ainda é assunto de debate e controvérsias. O atrelamento histórico da doença na infância com a SMD do adulto levou a um retrocesso em diversas áreas do conhecimento desta patologia, sendo talvez as questões relativas ao tratamento as mais afetadas. Dado a origem clonal da doença, postula-se que ela seja virtualmente incurável com as terapias convencionais. Muito se tem estudado a respeito do transplante de células-tronco hematopoéticas, nas suas mais diversas fontes e possibilidades, com alguns resultados promissores. O Grupo Cooperativo Brasileiro de Síndrome Mielodisplásica em Pediatria (GCB-SMD-PED) foi criado em 1997, com o objetivo de estudar esta doença na população brasileira, em seus mais diversos aspectos, quer sejam clínicos, epidemiológicos, citológicos, patológicos, citogenéticos ou moleculares. Após cinco anos de atividades, o Grupo Cooperativo iniciou discussões afim de propor protocolo terapêutico para suas crianças. Para tanto, como passo inicial, fez-se ampla revisão de literatura sobre o assunto, a qual é aqui discutida. Ainda com este fim, o grupo analisou a sobrevida dos pacientes matriculados no GCB-SMD-PED, os diagnósticos realizados na instituição de origem e as mais diversas abordagens terapêuticas seguidas, as quais variaram desde tratamento conservador, medidas de suporte, transfusões sangüíneas à transplante de medula óssea. Os autores descrevem as mais diferentes abordagens utilizadas para o tratamento da SMD em crianças, bem como o racional do emprego de cada modalidade terapêutica e os estudos pertinentes na área.

The management of Myelodysplastic Syndrome (MDS) in childhood is still a matter of debate and controversy. The historic relationship of this illness in children with MDS in adulthood delayed development in different areas, where perhaps knowledge related to treatment was the most affected. Due to the clonal origin of this illness, it is thought that it is virtually incurable with conventional therapies. There have been plenty of studies related to hematopoetic stem-cell transplantation with some promising results. The Brazilian Pediatric Myelodysplastic Cooperative Group (GCB-SMD-PED) was created in 1997, with the aim of studying this disease in the Brazilian population and its different aspects, whether clinical, epidemiological, cytological, pathological, cytogenetical or molecular. After five years of activities, the Co-operative Group has initiated discussions to propose therapeutic protocols for children. For this, as an initial step, a review of publications was performed about this subject, which is discussed in this article. The group also analysed the overall survival of patients referred to the Bra-PMD-CG and the different diagnostic and treatment schedules employed in each institution, varying from simply conservative treatment, palliative measures, blood transfusions to bone marrow transplantation. The authors describe the different ways used for MDS treatment in childhood, as well as the rationale employed of each therapeutic modality and the studies related to this area.