RESUMO
Cherubism is a rare hereditary fibro-osseous childhood disease characterized by bone degradation and fibrous tissue replacement at the angles of the mandible and at the tuberosity areas of the maxilla that leads to prominence of the lower face and an appearance reminiscent of the cherub's portrayal in Renaissance art. This disease has an autosomal dominant hereditary characteristic. The purpose of this report is to analyse laboratory tests, clinicopathological and radiographic features of cherubism and its intraoral manifestations in a patient during 4-years of follow-up, correlating the features observed in this case with those of the literature. Also discussed is the atypical and aggressive behaviour of this case during puberty.
Assuntos
Querubismo/fisiopatologia , Doenças Mandibulares/fisiopatologia , Adolescente , Querubismo/diagnóstico por imagem , Querubismo/patologia , Tecido Conjuntivo/patologia , Progressão da Doença , Assimetria Facial/diagnóstico por imagem , Assimetria Facial/patologia , Feminino , Seguimentos , Células Gigantes/patologia , Humanos , Doenças Mandibulares/diagnóstico por imagem , Doenças Mandibulares/patologia , Puberdade/fisiologia , Radiografia Panorâmica , Tomografia Computadorizada por Raios XRESUMO
Two cases of polymorphous low-grade adenocarcinoma of the papillary type, from minor salivary glands were studied by light microscopy and immunohistochemistry. One case exhibited a predominance of the papillary pattern, whereas the other presented the following patterns of histological appearance: papillary, solid, pseudocystic and tubular. Utilizing the peroxidase-antiperoxidase (PAP) method, the intermediate filament vimentin, keratin and S100 protein were observed in tumor cells. The immunohistochemical analysis revealed two types of neoplastic cells: myoepithelial and luminal.