Osteosarcoma of the mandible arising de novo in fibrous dysplasia. Case report
and review of the literature
Aikaterine TRIANTAFILLIDOU, Konstantinos PSOMADERIS, Fotis IORDANIDIS, Dimitris KARAKASIS
Hellenic Archives of Oral & Maxillofacial Surgery (2012) 1, 33-40
SUMMARY: Introduction: Malignant transformation of fibrous dysplasia is rare, accounting 4% of cases with McCune-Albright syndrome and 0,5% of monostotic type. Case presentation: An 11-year old boy presented with hard swelling and disfigurement of his mandible in conjunction with alterations of his dental occlusion. Fol - lowing biopsy, fibrous dysplasia was diagnosed. The patient underwent surgical contouring of the affected bone. Four years later a fast growing mass developed at the same site of the operated mandible. After an incisional biopsy the diagnosis of low-grade osteosarcoma was established. The patient underwent wide mandi - bulectomy and reconstruction of the defect with bone graft and is free of the disease for the last 14 years. Conclusion: Most cases of malignant transformation of FD are associated with irradiation of the fibrous dysplasia. The spontaneous transformation of the monostotic type is extremely rare. The development of a soft tissue mass or elevation of serum alkaline phosphatase in a patient with fibrous dysplasia should be considered alarming features and require further investigation to exclude sarcoma.
KEY WORDS: fibrous dysplasia, malignant transformation, osteosarcoma
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