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

Introduction: Chondromyxoid fibroma (CMF) is a rare benign bone tumor, usually arising in lower extremity long bone metaphysis.[1] They were first described in 1948 by Jaffe and Lichtenstein.[2] Their occurrence in the craniofacial region is exceptionally rare, with limited numbers of cases reported. Due to the paucity of cases, the diagnosis of CMF is challenging. Here, we summarize and analyze these cases as a whole and then separate them into calvarial and sinonasal categories, which seems to have clinical implications in terms of their symptomatology and treatment outcomes.

Methods: A systematic review on published cases of CMF from the year 1990 was conducted using the electronic databases PubMed, EMBASE, Scopus, and Web of Science. Cases of craniofacial CMF were identified from individual reports or from case series. Only reports published in English language describing CMF in humans were included. A PRISMA protocol was followed. Patient data that was collected included age, gender, clinical presentation, treatment, GTR rate when applicable, whether radiation was used, follow-up (months), and recurrence. Cases were divided into two groups: calvarial (involving flat skull bones) and sinonasal (including nasal cavity, ethmoids, sphenoid sinus, orbital cavity, sella or parasellar region, and clivus). The two groups were compared in terms of their clinical presentation and treatment outcomes.

Results: The systematic review identified 94 cases of craniofacial CMF including 30 and 64 cases in the calvarial and sinonasal groups respectively. In the comparison of the calvarial and sinonasal groups, the average age was found to be similar at 35 and 39 respectively. Both had a skew in gender ratio with female being more common in both, more significantly in the sinonasal group. The most common clinical presentation for patients with calvarial CMF was neurologic deficits followed by headaches and swelling. For patients with sinonasal CMF, the most common symptoms were nasopharynx symptoms such as nasal obstruction, difficulty breathing or swallowing, followed by vision changes and headache. Calvarial CMFs had a higher gross total resection (GTR) rate (85.7%) as compared with sinonasal group (67.6%). The overall recurrence rate was higher in the sinonasal group (25 vs. 11.1%) but surprisingly, the recurrence rate after GTR was lower in this group (4.3 vs. 12.5%). Radiation was used more frequently in the sinonasal group (15%) as compared with the calvarial group (4.5%).

Conclusion: The calvarial and sinonasal groups of craniofacial CMF are distinct diagnoses with important differences demonstrated with this data analysis. Sinonasal CMFs are less amenable for GTR owing to its complex location involving multiple compartments; however, a GTR in this location gives the best outcome with low recurrence rates.

• References

• 1 Wu CT, Inwards CY, O’Laughlin S, Rock MG, Beabout JW, Unni KK. Chondromyxoid fibroma of bone: a clinicopathologic review of 278 cases. Hum Pathol 1998; 29 (05) 438-446
• 2 Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol (Chic) 1948; 45 (04) 541-551