Desmoplastic fibroma of the temporal bone.

Background: Desmoplastic fibroma is a benign, locally aggressive, intraosseous neoplasm with a propensity for local recurrence. Desmoplastic fibroma most commonly originates within the mandible (70% of cases), and long bones with rare lesions reported in the maxillary, frontal, and parietal bones. We report two patients with desmoplastic fibroma arising within the temporal bone.

Study Design: Case report and literature review.

Patients: Two patients with desmoplastic fibroma with intracranial and extra cranial growth patterns are presented. Both were young females with aural fullness and decreased hearing.

Interventions: Diagnostic and therapeutic.

Results: Both tumors were surgically extirpated and the patients have remained disease free for 18-48 months. Neither tumor expressed estrogen or progesterone receptors.

Conclusion: Desmoplastic fibroma is a highly invasive local destructive lesion which is best treated by nondestructive surgical intervention. Immunohistochemical analysis may demonstrate hormonal receptors, in which case Tamoxifen may reduce recurrence.