Abstract
BACKGROUND: Primary pulmonary synovial sarcoma (PPSS) is an extremely rare pulmonary malignancy and often poses diagnostic challenges, especially in differentiating it from benign tumors. CASE PRESENTATION: A 46-year-old woman presented with a slowly enlarging, well-defined nodule in the right lower lobe of the lung. Positron emission tomography showed low FDG uptake (SUVmax 1.52), raising suspicion for a benign lesion. However, histopathologic examination following wedge resection revealed primary pulmonary synovial sarcoma. Subsequently, a right lower lobectomy was performed. The patient has remained recurrence-free for over one year. CONCLUSIONS: Surgical resection with clear margins is essential in the management of PPSS. This case highlights the difficulty in distinguishing PPSS from benign lung lesions based on imaging alone, underscoring the importance of pathological confirmation. Accumulation of more reported cases is needed to develop standardized treatment strategies.