Abstract
Primary pleuropulmonary synovial sarcoma (PPSS) is a rare malignant soft tissue sarcoma primarily affecting adolescents and young adults. Diagnosis relies on clinical examination, radiological imaging, and confirmation through histopathological and immunohistochemical analyses. Due to nonspecific symptoms, diagnosis is often delayed. Treatment typically involves a multimodal approach, including systemic chemotherapy, surgical intervention, and radiotherapy. We present the case of a 14-year-old female with a 5-month history of cough, low-grade fever, and weight loss. A contrast-enhanced chest CT scan revealed a large left thoracic mass with lung infiltration, mediastinal invasion, and multiple enlarged lymph nodes. Histopathological and immunohistochemical analyses confirmed a primary pulmonary synovial sarcoma positive for transducer-like enhancer of split-1 (TLE-1). The patient underwent 2 cycles of neoadjuvant chemotherapy with ifosfamide and doxorubicin, but no significant improvement was observed. Local control options, including surgery and radiotherapy, were deemed infeasible, and palliative care was initiated.