Malignancies in fibrous dysplasia

Abstract

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 sarcom 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. Cancer 1994; 73:1411–24.

Malignant Sarcomatous Transformation of Fibrous Dysplasia

“…The reported time interval between the initial presentation of FD and the initial presentation of malignant transformation ranges widely among case series. In some reports the diagnoses of FD and sarcoma were established concurrently [4].…”

“…The most frequent sites involved in malignant transformation are the craniofacial bones (46 %) and proximal femur (25 %). The humerus, pelvis, tibia and scapula are less commonly involved [4]. Warning signs that should alert clinicians to consider malignant transformation include rapidly increasing pain without apparent trauma, increase in serum alkaline phosphatase, or a significant rapid change in radiologic appearance [1,7].…”

Prognosis for craniofacial fibrous dysplasia after incomplete resection: age and serum alkaline phosphatase

“…It also has the potential to compress the optic nerve 17 . Malignant transformation is rare but may occur, most likely after radiation therapy with sarcomas being the most common histological type 9,16 .…”

McCune Albright syndrome in association with excessive GH secretion: case report

“…Treatment-related side effects include impaired glucose tolerance with use of long-acting somatostatin and gallbladder stones following combination treatment (12)(13)(14). In our patient, stone was observed in the gallbladder at the end of the one-year follow-up period, but fasting and postprandial blood glucose levels were found to be normal.…”