Ewing sarcoma of the Mandible: a systematic review and pooled survival analysis.
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BACKGROUND: Ewing sarcoma (ES) of the mandible is an extremely rare malignancy, representing less than 2% of all ES cases. Due to its low incidence, most available evidence derives from isolated case reports or small case series, resulting in limited knowledge regarding its clinical characteristics, management strategies, and prognostic factors. This study aimed to systematically review the literature on mandibular ES and to analyze survival outcomes and potential prognostic factors. METHODS: A systematic review was conducted according to PRISMA guidelines. PubMed/MEDLINE, Scopus, and the Cochrane Library were searched for studies reporting cases of mandibular ES. Case reports and case series providing extractable clinical and survival data were included. Demographic, clinical, and treatment-related variables were extracted. Continuous variables were summarized as medians and interquartile ranges (IQR). Overall survival (OS) was estimated using Kaplan-Meier analysis, and potential prognostic factors were explored through univariable Cox proportional hazards regression. RESULTS: A total of 38 studies comprising 59 patients were included. The median age was 12 years (IQR 7-16), and 49.2% of patients were male. Swelling was the most commonly reported presenting symptom, while pain was documented in 54% of cases. Most patients underwent multimodal treatment (83%), including chemotherapy, surgery, and/or radiotherapy. During follow-up, 21 deaths were recorded. Estimated OS rates at 1, 3, and 5 years were 82.5%, 70.6%, and 60.9%, respectively. The presence of pain at diagnosis was associated with significantly worse OS (HR 5.76, 95% CI 1.65-20.11; p = 0.01). Patients treated with single-modality therapy had poorer survival compared with those receiving multimodal treatment (HR 6.58, 95% CI 2.23-19.46; p < 0.005). Chemotherapy and surgery were associated with improved survival outcomes. CONCLUSIONS: Mandibular Ewing sarcoma is a rare but aggressive tumor that requires prompt diagnosis and a multidisciplinary treatment approach. Multimodal therapy, particularly the combination of chemotherapy and surgery, appears to provide the best survival outcomes. Pain at presentation may represent a negative prognostic factor. Further multicenter studies with standardized reporting and longer follow-up are needed to better define optimal management strategies and prognostic determinants.