Craniofacial Chondromyxoid Fibromas: A Systematic Review and Analysis Based on Anatomic Locations.

BACKGROUND: Craniofacial chondromyxoid fibromas (CMFs) are a rare benign tumor of cartilaginous origin. They are commonly misdiagnosed due to the paucity of information on tumor characteristics. We performed a systematic review to characterize CMFs located in different regions of the craniofacial skeleton.

METHODS: A search of the literature was executed using the search phrase "chondromyxoid fibroma" and included articles from 1990-2020. Sixty-eight articles met the inclusion criteria, with a total of 91 patients with analyzable data (22 with calvarial and 69 with sinonasal tumor locations). Descriptive analyses were performed to compare pre-selected characteristics between the 2 groups.

RESULTS: Sinonasal CMF frequently presented with cranial nerve palsy and expectedly had a high rate of nasal symptoms. Calvarial tumors frequently presented with an external mass and headache. Gross total resection (GTR) was achieved in a higher proportion of cases in the calvarial group versus the sinonasal group (83.3% vs. 53.1%). Overall recurrence rate at 17.7% was higher in sinonasal CMF compared with the calvarial tumors at 8.3%. Recurrences after GTR were similar in the sinonasal and calvarial groups (9.7% vs. 9.1%). In patients who did not achieve GTR, recurrence was higher in the sinonasal compared with the calvarial group (27.6% vs. 0%).

CONCLUSIONS: Craniofacial CMF in calvarial and sinonasal locations have distinct clinical characteristics and response to treatment. Sinonasal lesions tend to have higher recurrence compared to calvarial CMF. Performance of GTR is associated with decreased recurrence in all CMFs.