Condrossarcoma da base do crânio descoberto incidentalmente através de Síndrome de Maffucci
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Resumo
Introdução: A Síndrome de Maffucci (SM) é uma displasia mesodérmica não hereditária congênita associada a malformações venosas cutâneas com discondroplasia.
Objetivo: revisar a síndrome de MAffucci e apresentar um caso no qual o condrossarcoma assintomático da base do crânio foi diagnosticado como achado casual e tratado.
Resultados: Caracteriza-se por ser uma enfermidade rara com menos de 200 casos descritos pela literatura, não havendo estatísticas sobre sua epidemiologia e prevalência. A Síndrome de Maffucci tem como principal diagnóstico diferencial a Síndrome de Ollier. Outras patologias também devem ser pensadas diante do quadro apresentado, como o sarcoma de kaposi, síndrome de Klippel-Trenaunay-Weber,síndrome de Proteus e doença de Gorham.
Conclusão: deve-se ter alto grau de suspeita de lesões da base do crânio em pacientes com síndrome de maffucci.
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