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Malignancies in fibrous dysplasia
P Ruggieri1, F H Sim, J R Bond
1Department of Orthopedics, Mayo Clinic, Rochester, MN 55905.
Background:
Malignancies in fibrous dysplasia are rare. Most cases have been published as single case reports. The role of radiation therapy in the occurrence of sarcoma in fibrous dysplasia is still controversial.
Methods:
The Mayo Clinic files were reviewed, including Mayo Clinic cases and consultation cases, to collect all cases of sarcomas arising in fibrous dysplasia.
Results:
Among 1122 cases with a histologic diagnosis of fibrous dysplasia, 28 cases of sarcoma were found. These 28 cases included 16 Mayo Clinic cases and 12 consultation cases. The sarcomas occurred in 19 cases of monostotic fibrous dysplasia and 9 cases of polyostotic disease (only 1 of Albright's syndrome). The most common histotype was osteosarcoma (19 cases), followed by fibrosarcoma (5 cases), chondrosarcoma (3 cases), and malignant fibrohistiocytoma (1 case). Of the 28 patients, 13 (46%) had received radiation therapy before the sarcoma developed. Most of these sarcomas occurred in the craniofacial bones (13 cases) or in the proximal femur (7 cases), followed by the humerus, pelvis, tibia, and scapula.
Conclusions:
Prognosis was poor. Sarcomas may arise with or without radiation. Early diagnosis and adequate treatment may lead to improved prognosis.