Aneurysmal bone cyst (ABC) is a benign locally destructive bone neoplasm composed of multi-loculated blood-filled cystic spaces. The most common sites of involvement are the meta-diaphysis of the long bones and posterior elements of the vertebrae. Secondary, ABC-like changes can complicate a variety of other benign and malignant primary bone neoplasms, including giant cell tumor, fibrous dysplasia, and osteosarcoma. About two-third of primary ABCs have a rearrangement of the USP6 gene, which is not present in the ABC-like changes that occur secondary to other primary bone tumors (i.e., secondary ABC). Primary ABC of bone carries a variable but generally high rate of local recurrence. This paper provides an overview of the pathophysiology, clinical presentation, radiographic and pathologic findings, treatment, and prognosis of ABC.
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We report a case of nasal chondromesenchymal hamartoma. A 14-year-old boy presented with a 5 cm sized mass in the left maxillary sinus, facial swelling and a loose tooth. A subtotal left maxillectomy with a bone graft was performed.
The excised mass was composed of partly encapsulated, solid and cystic fragments of soft tissues. The mass contained chondroid and myxoid areas consisting of mesenchymal tissues including hyaline cartilage, osteoid and spindle cells in various proportions. The hyaline cartilage component was the most prominent. The spindle cell component had a fibrous matrix with variable myxoid or sclerotic changes. Thick hyalinized eosinophilic osteoid-like trabeculae were focally present. Immunohistochemically, all the mesenchymal cells tested positive for vimentin. The chondrocytes tested positive for the S-100 protein, and the spindle cell component showed focal immunoreactivity for smooth muscle actin and desmin. However, the cells were negative to pan-cytokeratin and p63.