Abstract
Synovial sarcoma, a malignant mesenchymal tumor, is primarily associated with the extremities. Nevertheless, its appearance within the head and neck region, particularly in the maxillary area, is remarkably rare. This rarity underscores the significance of each case in unraveling the complexities of its behavior and management strategies. The core focus of this research is a detailed case report involving a 6-year-old female patient who presented with a conspicuous swelling in the left posterior maxilla. Subsequent incisional biopsy led to microscopic identification of malignant spindle cell proliferation, marked by dysplastic changes, and abundant mitoses. Immunohistochemical (IHC) analysis demonstrated negative reactivity for neural and muscular markers, while positive expression of Vimentin, Bcl-2, and TLE1. These morphological and IHC findings coalesced to definitively diagnose synovial sarcoma, substantiated by a notable 40% Ki67 proliferative index. The chosen treatment strategy encompassed surgery and radiotherapy, which yielded successful outcomes, with no recurrence observed during the one-year follow-up period. Beyond the specific case, this article undertakes a review of existing literature, meticulously analyzing nine similar cases reported in scholarly sources.