Fibrous dysplasia of occipital and temporal bone. A case report

Abstract

Fibrous dysplasia is a rare non-malignant condition where fibrous tissue replaces the normal bone architecture. Involvement of temporal and occipital bones is exceptionally rare and is associated with unique complications. A 10-year-old boy presented with right retroauricular enlargement and pain. Imaging studies and biopsy revealed fibrous dysplasia of the temporal and occipital bones. There was no hearing loss or sequelae arising from posterior fossa compression. The patient was discharged with follow-up instructions. Only 10 cases of occipital bone fibrous dysplasia have been reported in the medical literature. Occipital bone fibrous dysplasia can be complicated with Chiari malformation and syringomyelia while temporal bone involvement is associated with hearing loss. These potential developments require close follow-up that includes detailed neurologic examination, imaging and audiology.

A Rare Case of a Primary Leiomyoma of the Clivus in an Immunocompetent Patient and a Review of the Literature Regarding Clival Lesions

“…Fibrous dysplasia is also characterized by a high frequency of activating mutations of the GNAS gene, which encodes the stimulatory G-protein αsubunit (Gsα) [35]. It has an incidence of 1 per 30,000 [36], but fibrous dysplasia in the clivus is a casuistic incident. There are three clinical patterns of these lesions.…”