RESUMO
We present a patient with neurofibromatosis type 1, with the clinical, radiological and histological features of cherubism mandibular lesions, and multiple osteolytic, geographic lesions in both femurs, consistent with multiple non-ossifying fibromas. We have been unable to find a similar case in the world literature. We discuss our findings in relationship with a number of syndromes that present clinical, radiological or pathological similarities.
Assuntos
Querubismo/complicações , Neurofibromatose 1/complicações , Osteólise/etiologia , Querubismo/diagnóstico , Criança , Diagnóstico Diferencial , Fêmur , Humanos , Masculino , Neurofibromatose 1/diagnóstico , Osteólise/diagnósticoRESUMO
No disponible
Assuntos
Humanos , Cordoma/patologia , Neoplasias Ósseas/patologia , Notocorda/patologia , Biópsia por Agulha Fina/métodosRESUMO
No disponible
Assuntos
Humanos , Cordoma/patologia , Neoplasias Ósseas/patologia , Notocorda/patologia , Biópsia por Agulha Fina/métodosRESUMO
El término condrosarcoma es usado para describir ungrupo heterogéneo de tumores con características morfológicasy comportamientos clínicos diversos. Se asume quelos condrosarcomas son tumores malignos constituidos porcélulas de fenotipo cartilaginoso que usualmente muestranun patrón histológico lobulado y tienden a mantener sunaturaleza esencialmente cartilaginosa a lo largo de su evolución,independientemente de la localización del tumor,tanto en los tumores esqueléticos como extraesqueléticos.Se reconocen una serie de variantes, de características morfológicas,radiológicas y clínicas diferentes. En esta revisiónse exponen las referidas características de las diferentesvariantes de condrosarcoma, y las dificultades diagnósticaspara el patólogo, haciendo énfasis en los criterios parasu diagnóstico diferencial, incluyendo los nuevos conocimientosde citogenética y patología molecular
The term chondrosarcoma is used for describing a heterogeneousgroup of tumors with diverse morphological featuresand clinical behaviour. It is assumed that chondrosarcomasare malignant tumors composed of cells with a cartilaginousphenotype that usually show a lobulatedhistological pattern and tend to maintain their essentiallycartilaginous nature along their evolution, independently ofthe site of the tumor, as well in osseous as in extra skeletaltumors. Several variants of chondrosarcomas are recognized.They have different morphological, radiological andclinical features. In this revision the above mentioned featuresof the different variants and the diagnostic difficultiesfor the pathologist are commented, and emphasis is given tothe criteria for its differential diagnosis, including recentknowledge about cytogenetics and molecular biology