Hereditary multiple exostosis is the most common form of bone dysplasia. This entity is also known as diaphyseal aclasis, hereditary deforming chondrodysplasia, multiple hereditary exostoses, multiple osteochondromatosis, multiple cartilaginous exostosis, dyschondroplasia, and Ehrenfried disease. It is an inherited autosomal dominant disease with predominance in males and a benign condition characterized by the presence of multiple exostosis or osteochondromas arising from long and flat bones.Osteochondroma is the most common benign tumor in persons between 10 and 30 years of age. It accounts for 20% to 50% of all benign tumors and 10% to 15% of all bone tumors. It is more commonly located at the level of the metaphysis of long bones. However, osteochondroma is rare at the level of the facial bones and skull base. It has been reported in the maxillary sinus and in different parts of the mandible, such as the condyle, ramus, body, and symphyseal region. It is very uncommon in the coronoid process and occipital bone.Jacob disease, or osteochondroma of the mandibular coronoid process, is a benign skeletal tumor that is rare in the oral and maxillofacial skeleton. A review of the literature revealed only 41 histologically proven cases of 52 reported cases. To the best of the authors' knowledge this is the first clinical report of bilateral coronoid osteochondroma and associated occipital exostosis in a patient with hereditary multiple exostosis.
Central Odontogenic Fibroma is a benign neoplasm of mesenchymal origin that makes up less than 5% of odontogenic tumors. There is a variation of this lesion that includes a zone of giant cells. This neoplasm is characterized by fibroblast proliferation, a component of apparently inactive odontogenic epithelium within a mature connective tissue stroma and multinucleated osteoclast-like giant cells. Clinically, it manifests as a slow-growing central lesion, which causes painless cortical expansion and may occasionally cause the displacement of adjacent dental organs or resorption. We present a new case of this combined lesion that was revealed radiographically as a well-defined radiolucent area in the left mandibular body. Histologically, we identified a mesenchymal lesion composed of mature fibrocellular tissue, where islands and cords of odontogenic epithelium and fibrocellular areas with numerous giant cells can be distinguished. The immunohistochemical examination was positive for giant cells with the marker CD68 and positive for epithelium cords with the marker CK19, which indicates that this an odontogenic lesion.
Las lesiones del clivus, vértebras cervicales altas y la unión cervicomedular frecuentemente representan dificultades para el neurocirujano. Los abordajes quirúrgicos que están normalmente disponibles proveen menor exposición de la óptima tanto para remover la lesión primaria como para reparar la duramadre. El abordaje transoral que combina la glosotomía medial labiomandibular, hendidura del paladar blando y resección del paladar duro se usó en un caso expuesto aquí. Esta técnica no sólo produjo la exposición transversa y sagital máxima, sino que redujo considerablemente la distancia operatoria entre el neurocirujano y la unión cervicomedular.
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