Oral and Maxillofacial Low-Grade Myofibroblastic Sarcoma: A Systematic Review

Abstract

Background: This systematic review (SR) aimed to summarize the clinical, histopathological, and immunohistochemical features of low-grade myofibroblastic sarcoma (LGMS) in the oral and maxillofacial region (OMR), as well as treatment protocols, recurrence, follow-up, and metastasis rates. It follows PRISMA 2022 guidelines and is registered in PROSPERO (CRD42023409758).

Methods: An electronic search included PubMed, EMBASE, Scopus, Web of Science, LILACS, Google Scholar, and ProQuest databases, and bias risk was assessed using the Joanna Briggs Institute tool. Statistical analyses, including Fisher's and Chi-Squared tests, were conducted to explore associations between clinical variables, and survival analysis was performed using the Kaplan-Meier method.

Results: Forty-three studies covering 78 cases were included. LGMS showed a slight female predominance (51.28%) and an average patient age of 35.35 years. The most affected sites were the mandible (29.4%), maxilla (19.23%), and tongue (15.38%). Clinical presentations included masses (30.76%), tumors (26.92%), and swelling (12.82%), with symptom duration ranging from 1 week to 30 months. Surgery alone was the primary treatment (60.25%), with recurrence in 20.51% and metastasis in 5.12% of cases. One-year and 5-year overall survival rates were 92.6% and 71.8%, respectively. Surgery as the sole treatment was significantly associated with a lower metastasis rate (p = 0.005).

Conclusion: Our findings highlighted the importance of considering LGMS in differential diagnoses of myofibroblastic lesions and underscored the need for a comprehensive immunohistochemical analysis. Complete surgical excision remains the preferred treatment, though long-term follow-up is needed to better understand recurrence and metastasis risks.

Keywords: Low-grade myofibroblastic sarcoma; Oral and maxillofacial region; Prognostic; Survival.