Radiological Patterns in Maxillofacial Ewing Sarcoma: A systematic review with pooled patient-level descriptive analysis

Background

Ewing sarcoma (ES) is a rare malignant bone tumor with predilection for the mandible and maxilla in the head and neck region. However, existing literature comprises fragmented case reports and small case series that fail to establish consolidated, evidence-based understanding of characteristic radiological patterns in the maxillofacial region, hindering timely diagnosis and potentially leading to misdiagnosis or delayed intervention.

Methodology

A systematic review and pooled patient-level descriptive analysis were conducted according to the PRISMA guidelines and pre-registered on PROSPERO. Comprehensive searches of PubMed, OVID, and Cochrane databases (inception to July 2025) identified studies reporting radiological findings of biopsy-confirmed maxillofacial Ewing sarcoma. Quality assessment using Joanna-Briggs Institute criteria ensured inclusion of only high-quality cases (quality score ≥4/5). Synthesis Without Meta-analysis (SWiM) methodology with pooled prevalence estimation and binomial vote-counting analysis were employed for 68 published cases.

Results

Four radiological features demonstrated consistent predominance across pooled cases: soft tissue mass presence (100%, 95% CI: 94.7–100.0%), enhancing soft tissue (77.6%, 95% CI: 65.8–86.9%), cortical destruction (69.0%, 95% CI: 55.5–80.5%), and notably, absence of periosteal reaction (84.7%, 95% CI: 73.0–92.8%). Location-specific radiological phenotypes were evident: maxillary tumors demonstrated near-universal sinus involvement (100%) with high soft tissue enhancement (92.3%), whereas mandibular tumors showed predominant cortical destruction (80.0%) and teeth involvement (81.2%).

Conclusion

MRI and CT are essential for characterizing the distinctive radiological profile of maxillofacial Ewing sarcoma, enabling early identification and improving patient outcomes in this rare malignancy.

Highlights

• First pooled review to summarize imaging features of maxillofacial Ewing sarcoma

• Analysis of 68 published cases reveals consistent imaging patterns.

• Most tumors show soft tissue mass and bone damage without surface reaction.

• Jaw tumors differ from long bone tumors in their imaging appearance.

• Upper and lower jaw tumors show distinct location-specific features.