Mesenchymal chondrosarcoma of the maxilla: Case report and clinicopathologic review

AbstractMesenchymal chondrosarcoma is extremely rare, accounting for around 1% of all chondrosarcomas. It occurs in the axial skeleton, the head and neck region and a high proportion of extraskeletal sites. Fewer than 40 cases of mesenchymal chondrosarcoma in the maxilla have been reported in English literature. The neoplasm is aggressive, with a high tendency for late recurrence and delayed distant metastasis. Here we present a 50-year-old man with palatal mucosal swelling for 6 months. The initial clinical impression was a benign minor salivary gland neoplasm. The biopsy revealed hyaline cartilage, alternating with undifferentiated small round cells, pathognomonic for mesenchymal chondrosarcoma. Diagnosis of biopsy specimens remains a challenge because of variable tumor components, and specimens may contain only one of the two neoplastic elements. The patient received radical surgery and has been disease free for 40 months. Clinicians and pathologists should be aware of this entity in the differential diagnosis of maxillofacial neoplasms, to prevent misdiagnosis and delayed intervention