2017 Volume 110 Issue 11 Pages 765-771
Patients with fibrous dysplasia in the craniofacial region are usually followed up, in case there are no problems or pain. In very rare cases, fibrous dysplasia undergoes malignant transformation. Herein, we report a case of osteosarcoma of the temporal region that arose in a region of fibrous dysplasia.
A 60-year-old man consulted our hospital with a chief complaint of pain and swelling of his right temple. He had previously undergone surgery at other hospitals for fibrous dysplasia at multiple sites of the body, including the maxilla, rib, femur and humerus. CT revealed tumors that appeared to be fibrous dysplasia, located within and continuous with the maxilla, while a tumor with bone destruction was observed in the right temporal and cheek bones. Biopsy revealed the diagnosis of osteosarcoma. Skull base surgery was performed after administration of chemotherapy with cisplatin and doxorubicin.
The right eyeball was preserved and the tumor was excised en bloc. However, we could not obtain a sufficient margin. Histopathological examination of the resected specimen revealed that the preoperative chemotherapy had had little effect and that the surgical margin was positive.
Radiotherapy and chemotherapy were administered as postoperative adjuvant treatments. One year eight months later, the tumor relapsed near the clivus.
Although it is rare, patients with fibrous dysplasia should be made aware of the possibility of malignant transformation of the lesion after a prolonged period and should immediately consult their physician in the event that they experience pain.
