RESUMO
Mesenchymal chondrosarcomas are rare malignant tumours in children, especially, in neonates. The authors present a case of congenital mesenchymal chondrosarcoma in a 1-day neonate located in sacrum. According to the authors' literature searches, this case is the first congenital sacral mesenchymal chondrosarcoma. We also reviewed the papers published in English literatures.
Assuntos
Condrossarcoma Mesenquimal/diagnóstico , Sacro , Neoplasias da Coluna Vertebral/diagnóstico , Condrossarcoma Mesenquimal/terapia , Terapia Combinada , Diagnóstico Diferencial , Evolução Fatal , Humanos , Recém-Nascido , Masculino , Neoplasias da Coluna Vertebral/terapia , Tomografia Computadorizada por Raios XRESUMO
Hypertrophic pyloric stenosis (HPS) is the most common gastrointestinal obstructive disease in infants which requires surgery. Typical presentation is at 4-6 weeks of life but can present very earlier or congenitally. Here we present 3 cases of HPS which presented in the first day of life with co-existing malrotation, annular pancreas, and duodenal atresia. The exact etiology of HPS is not fully understood, however, genetic and maternal factors, hormonal factors, abnormalities of various components of the pyloric muscle, growth factors, extracellular matrix elements, nerve and ganglion cells synapses, nerve supporting cells, neurotransmitters and interstitial cells, drugs, and feeding have been implicated. In the HPS concomitant with a distal obstruction, mean that these obstructions may role in pathogenesis of HPS or may exacerbate other factors to produce HPS in a very early age or congenitally. The purpose of this manuscript is to present three cases of HPS in the first day of life with other GI anomalies. We hypothesize that the etiology of some cases of HPS may be secondary to distal intestinal obstructions especially when it presents very early in life, or at least to concern a distal obstructions in this very early presentation.