Chondromyxoid fibroma of the temporal bone

BACKGROUND Chondromyxoid fibromas are benign neoplasms comprising approximately 0.5% of primary bone tumors. The occurrence of this tumor in the skull is extremely rare, with only four previously reported cases involving the temporal bone and a total of 17 cases with intracranial involvement. CASE DESCRIPTION We describe the case of a 22-year-old man who experienced a 1-year history of headaches with progressive ear pain and mild hearing loss. Computed tomography and magnetic resonance imaging of the head demonstrated an extraaxial tumor arising from the left temporal mastoid region. A craniotomy was performed and the tumor resection required extensive drilling of the temporal bone. Grossly, the tumor consisted of a firm semigelatinous myxomatous tissue containing multiple areas of calcification and was histologically consistent with chondromyxoid fibroma. CONCLUSIONS Complete resection is the goal of surgery. Recurrence rates of 7% to 27% have been reported, dependent on the degree of initial resection. Radiation is not recommended because of the potential for sarcomatous conversion. Reoperation may be indicated for symptomatic recurrence.