RESUMO
The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw, multiple basal cell nevi carcinomas and skeletal abnormities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the KCOTs are usually one of the first manifestations of the syndrome. This article paper reports the case of a patient, a 10-year-old boy with NBCCS, emphasizing its clinical and radiographic manifestations. This study highlights the importance of health professionals in the early diagnosis of NBCCS and in a preventive multidisciplinary approach to provide a better prognosis for the patient.
Assuntos
Diagnóstico Precoce , Síndrome do Nevo Basocelular/diagnóstico , Biópsia , Criança , Diagnóstico Diferencial , Humanos , Masculino , Tomografia Computadorizada por Raios XRESUMO
Primary intraosseous carcinoma of the mandible is rare, and when it occurs often arises within or closely associated with an odontogenic cyst. The purpose of this article is to show the role of computed tomography (CT) in the early detection of carcinomatous changes, because of its specificity in establishing the nature of the lesion. An unusual case of a squamous cell carcinoma that arose in an odontogenic cyst is described. The appearance on the panoramic radiograph was suggestive of a benign cystic lesion in the left side of the mandible, but when a CT scan was performed, the appearance of the lesion was compatible with malignancy. Histopathological analysis confirmed the suspicion of a squamous cell carcinoma, but without CT investigation it would be impossible to demonstrate that carcinomatous change had developed from a cystic lesion.
Assuntos
Carcinoma de Células Escamosas/diagnóstico por imagem , Doenças Mandibulares/patologia , Neoplasias Mandibulares/diagnóstico por imagem , Cistos Odontogênicos/patologia , Idoso , Carcinoma de Células Escamosas/patologia , Transformação Celular Neoplásica/patologia , Feminino , Humanos , Neoplasias Mandibulares/patologia , Radiografia Panorâmica , Tomografia Computadorizada por Raios XRESUMO
The objective was to investigate two cases of solitary fibrous tumor (SFT) of oral mucosa, emphasizing the differential diagnosis with one case of oral hemangiopericytoma (HPC), in terms of their morphological and immunohistochemical features. Solitary fibrous tumors showed cellularity and collagenization varying from area to area, focal perivascular hyalinization, scattered giant nuclei cells and abundant mast cells throughout the tumor. The hemangiopericytoma case exhibited thin-walled and dilated vessels lined with flat endothelial cells, identified by "staghorn appearance". Tumoral cells of solitary fibrous tumor exhibited immunohistochemical positivity for CD34, as well as endothelial cells. The hemangiopericytoma was positive only in endothelial cells. In solitary fibrous tumor, alpha-smooth muscle actin, h-caldesmon and laminin stained the wall vessels. In hemangiopericytoma, on the other hand, the wall vessels were positive only for laminin, which staining was also observed in perivascular tumoral cells. The morphological and immunohistochemical differences observed allowed us to infer these lesions constitute distinct entities.