RESUMO
Twenty-seven infants under one year of age with metastatic neuroblastoma were studied for osteomedullary metastases. They were evaluated by at least two imaging procedures: X-ray films and MIBG scan. Taking into account the results of these investigations, 3 groups were defined: osteomedullary metastases were detected in 8 infants by both X-ray and MIBG scan, no osteomedullary sites were detected by either technique in 13 patients, and 6 had osteomedullary foci with positive MIBG uptake but no radiological abnormality. These three groups were apparently different in terms of median age, response to chemotherapy, and long-term survival. The third group, which has not been previously described, appears to have a better prognosis than patients with radiologically detectable bone lesions. Nevertheless it was necessary to use intensive chemotherapy to obtain remission in these particular patients. It should be mandatory to specify the investigatory technique used to describe osteomedullary metastases in infants and treatment should be adapted according to this grouping system.
Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/secundário , Radioisótopos do Iodo , Iodobenzenos , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/secundário , 3-Iodobenzilguanidina , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/mortalidade , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Neuroblastoma/tratamento farmacológico , Neuroblastoma/mortalidade , Prognóstico , Cintilografia , Indução de Remissão , Taxa de Sobrevida , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
From 1960 to 1987, 22 neonates were treated at the Institut Gustave-Roussy for a Pepper' syndrome. During this period of time, 83 children were treated for the same syndrome, the neonatal type representing 26% of cases. Twelve of the 22 neonates presented with the rapidly evolving type, requiring an immediate aggressive treatment. In 4 of them death occurred early despite treatment. The 10 other neonates presented with a slowly progressive disease. In 5 of them treatment of the metastatic disease was not necessary to obtain remission. The survival rate of disease without relapses in 77%, with a median follow-up of 8 years. So, despite the high incidence of rapidly progressive forms, the prognosis of this disease remains favorable in neonates, provided an accurate treatment initiated early.
