J Korean Cleft Palate-Craniofac Assoc.  2006 Oct;7(2):87-90.

Clinical Experience of Malignant Transformation of Multiostotic Fronto-Orbital Fibrous Dysplasia

• Kim HK
• Seo YM

Abstract

The malignant transformation of fibrous dysplasia is a rare condition and the incidence is estimated at 0.4% for fibrous dysplasia and 4% for Albright's syndrome. And fronto-orbital fibrous dysplasia can have a devastating outcome with high rates of recurrence and blindness, especially following incomplete resection. So a radical and extensive approach to treatment is recommended. We report the case of a multiostotic fronto-orbital fibrous dysplasia in a 54-year-old male patient which had a malignant transformation to osteosarcoma. There had been a recurrence after partial removal and no effect by chemotherapy. Although we had have a favorable outcome in functional and aesthetic aspects without any evidences of recurrence by total removal and reconstruction with titanium mesh, the patient died with lung metastasis 4 and half years later after the first operation.

Keyword

Fibrous dysplasia; Malignant transformation; Reconstruction

MeSH Terms

Blindness
Drug Therapy
Fibrous Dysplasia, Polyostotic
Humans
Incidence
Lung
Male
Middle Aged
Neoplasm Metastasis
Osteosarcoma
Recurrence
Titanium
Titanium