Primary Pulmonary Synovial Sarcoma: A Case Report and Review of Current Diagnostic and Therapeutic Standards

Abstract

Abstract Learning Objectives After completing this course, the reader will be able to: Describe primary pulmonary synovial sarcoma as a rare tumor that usually arises in young adults as a large pleural-based intrathoracic mass. Explain that the diagnosis is made by histology, immunohistochemical studies, and, more recently, by identification of the SYT-SSX1 or SYT-SSX2 chromosomal translocations. List factors that portend a worse prognosis, including inability to achieve a complete resection, larger tumor size, male sex, older age, high-grade tumor with necrosis, and the SYT-SSX1 variant. Access and take the CME test online and receive 1 hour of AMA PRA category 1 credit at CME.TheOncologist.com A 30-year-old female presented with hemoptysis, chest pain, and a rapidly enlarging pleural-based mass, and was found to have primary synovial sarcoma of the lung. Primary pulmonary sarcomas comprise <1% of all primary lung malignancies. They present clinically in young adults with cough, chest pain, shortness of breath, or hemoptysis, with a mass on x-ray and computerized tomography scan. Diagnosis is made by histology and immunohistochemistry. Histologic diagnosis has recently been supplemented by cytogenetic analysis, which offers important prognostic information. The mainstay of treatment remains complete surgical excision. Prognosis is poor, with an overall 5-year survival rate of 50%.