Assuntos
Humanos , Masculino , Lactente , Neoplasias Cutâneas/congênito , Neoplasias Faciais/congênito , Miofibromatose/congênito , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Neoplasias Faciais/diagnóstico , Neoplasias Faciais/patologia , Miofibromatose/diagnóstico , Miofibromatose/patologiaRESUMO
Abstract: Infantile myofibromatosis is a mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle and viscera. It is the most common fibrous tumor in childhood. We present a newborn with skin and bone disease without visceral involvement, who showed good response to vinblastine and methotrexate. Clinical features, etiology, diagnosis, and treatment are reviewed.
Assuntos
Humanos , Masculino , Recém-Nascido , Miofibromatose/congênito , Vimblastina/uso terapêutico , Imuno-Histoquímica , Metotrexato/uso terapêutico , Resultado do Tratamento , Miofibromatose/patologia , Miofibromatose/tratamento farmacológico , Fármacos Dermatológicos/uso terapêuticoRESUMO
La miofibromatosis infantil es una enfermedad del período neonatal o de la infancia temprana; consiste en la aparición de uno o múltiples tumores fibrosos que afectan piel, tejido celular subcutáneo, músculos, huesos y/o vísceras. Son tumores con tendencia a la involución espontánea, y su pronóstico está condicionad por la presencia o no de compromiso visceral. Se presenta el caso de un recién nacido de 48 horas de vida, con una tumoración en miembro superior derecho, sin compromiso visceral, que presentó involución espontánea en un plazo de 8 meses.
Infantile myofibromatosis is an illness of the neonatal period or early childhood. It consists of the appearance of one or more fibrous tumors which affect the skin, subcutaneous tissue, muscles, bones and/or viscera. These tumors tend to undergo spontaneousinvolution, and the prognosis is conditioned by the presence or absence of visceral liability. The case of a 48-hour newborn is described. The infant presented a tumour in the upper right member, which did not revealedvisceral liability and which resulted in spontaneous involution over an eight month span.