This is the first report (to our knowledge) that describes a patient with massive polyostotic fibrous dysplasia involving the calvaria and facial skeleton that subsequently underwent transformation to a malignant mesenchymoma with elements of chondrosarcoma, osteosarcoma, and rhabdomyosarcoma arising in the maxilla. Malignant transformation occurred in the absence of prior radiation exposure, osteomyelitis, or known bony infarction. A review of the literature did not reveal any similar cases of massive fibrous dysplasia of the maxilla degenerating to multiple simultaneous malignant histotypes.
Arch Otolaryngol Head Neck Surg. 1997;123:106-109
Beuerlein ME, Schuller DE, DeYoung BR. Maxillary Malignant Mesenchymoma and Massive Fibrous Dysplasia. Arch Otolaryngol Head Neck Surg. 1997;123(1):106-109. doi:10.1001/archotol.1997.01900010116019