Abstract
BACKGROUND: Sarcomas of the head and neck region are rare tumors, constituting less than 1% of malignant neoplasms in this area, of which few cases (20%) originate from bone or cartilage. Chondrosarcoma is a malignant neoplasm that develops in bone, with a predilection for the pelvis, chest wall, and scapula, and is uncommon in the maxilla and jaw. Although this type of lesion has locally aggressive behavior, destroying the affected bone, it can metastasize when it is not diagnosed early and compromise the patient's life. CASE SUMMARY: On intraoral examination of a 32-year-old female with a tumor in the middle third of the face, a well-defined rise in volume of approximately 3 cm in diameter was observed. Computed tomography with 3-dimensional reconstruction was performed, and we observed that the osteolytic lesion affected the vestibular cortex as the palatal bone. Hematoxylin and eosin staining revealed an appearance that was similar to mature hyaline cartilage, hypercellularity, nuclear and cellular pleomorphism, and multinucleated cells, with significant vacuolization. CONCLUSION: Determination of the clinical and histopathological characteristics of rare neoplasms in the maxillofacial region, such as chondrosarcomas, allows the pathologist and surgeon to make the appropriate therapeutic decisions, optimizing the patient's prognosis.