Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Antineoplastic Agents/administration & dosage , Dactinomycin/administration & dosage , Heart Atria/pathology , Kidney Neoplasms/drug therapy , Vena Cava, Inferior/pathology , Vincristine/administration & dosage , Wilms Tumor/drug therapy , Chemotherapy, Adjuvant , Kidney Neoplasms/mortality , Kidney Neoplasms/pathology , Kidney Neoplasms/surgery , Neoplasm Invasiveness , Neoplasm Staging , Retrospective Studies , Survival Analysis , Time Factors , Wilms Tumor/mortality , Wilms Tumor/pathology , Wilms Tumor/surgeryABSTRACT
OBJECTIVES: The aim of this retrospective study is to compare surgical complications and long-term survival in children with Wilms' tumor (WT) and tumor thrombus receiving or not preoperative chemotherapy MATERIALS AND METHODS: Review of the charts of 155 children with WT treated between 1983 and 2005, and analysis of 16/155 (10.3 percent) children with WT who presented cavoatrial tumor extension, being 8/16 IVC and 8/16 atrial thrombus RESULTS: Median age was 54 months. 2/16 had cardiac failure as the first symptom. 11/16(7 IVC and 4 atrial extension) (67 percent) were submitted to preoperative chemotherapy with vincristine plus actinomycin D, and 5/16(1 IVC and 4 atrial) (33 percent) underwent initial nephrectomy and thrombus resection. So, 11 patients were submitted to preoperative VCR/ACTD and 2/11 (18.1 percent) had complete regression of the thrombus, 6/11(54.5 percent) partial regression and 3/11 (27 percent) had no response. Among the partial responders, nephrectomy with thrombus removal was performed in all, including one patient with previous intracardiac involvement, without extracorporeal circulation procedures. In two of the three non-responders, cardiopulmonary bypass was necessary for thrombus removal. There were no surgical related deaths. Long-term survival is 91 percent in the group submitted to preoperative chemotherapy and 100 percent in the group who had surgery as first approach CONCLUSION: Preoperative chemotherapy was able to reduce thrombus extension in 8/11 (73 percent) treated patients and cardiopulmonary bypass was avoided in 2 patients with atrial thrombus. Surgical resection of tumor and thrombus was successful in all cases, receiving or not preoperative chemotherapy and overall survival was similar in both groups.
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Antineoplastic Agents/administration & dosage , Dactinomycin/administration & dosage , Heart Atria/pathology , Kidney Neoplasms/drug therapy , Vena Cava, Inferior/pathology , Vincristine/administration & dosage , Wilms Tumor/drug therapy , Chemotherapy, Adjuvant , Kidney Neoplasms/mortality , Kidney Neoplasms/pathology , Kidney Neoplasms/surgery , Neoplasm Invasiveness , Neoplasm Staging , Retrospective Studies , Survival Analysis , Time Factors , Wilms Tumor/mortality , Wilms Tumor/pathology , Wilms Tumor/surgeryABSTRACT
Objetivo: apresentar a experiência do Serviço de Cirurgia Pediátrica do Instituto da Criança do HCFMUSP no diagnóstico e tratamento de crianças com anomalias do arco aórtico e definir a importância dos exames complementares para o diagnóstico. Método: estudo retrospectivo de 22 crianças com diagnóstico de compressão traqueoesofágica por anel vascular tratadas no Instituto da Criança, no período de 1985 a 2000, investigando-se dados clínicos pré e pós-operatórios, exames complementares e evolução.Resultados: a anomalia vascular mais freqüente foi artéria inominada direita anômala (10 casos), seguido de duplo arco aórtico (7 casos) e arco aórtico à direita (5 casos). Os sintomas predominantes foram respiratórios (86por cento) e de início precoce (76por cento desde o período neonatal). Entretanto, o diagnóstico definitivo na maioria dos casos (60por cento) só foi estabelecido após l ano de vida. O exame mais importante para o diagnóstico foi o esofagograma. A correção de todas anomalias foi realizada por toracotomia póstero-lateral esquerda. Não ocorreram complicações cirúrgicas. A evolução foi pior nos casos operados mais tardiamente. Todas as crianças permanece- ram sintomáticas por até 6 meses, apesar de significativa melhora no pós-operatório. Conclusão: o diagnóstico de anel vascular deve ser investigado nas crianças com sintomas respiratórios de início precoce e nas "chiadoras'. de difícil controle. O diagnóstico pode ser realizado de forma simples através do esofagograma. Os demais exames de imagem acrescentam poucas informações e são dispensáveis na maioria dos casos. Os sintomas respiratórios podem persistir com menor intensidade por períodos variáveis no pós-operatório