Rapid metastatic progression of primary pulmonary synovial sarcoma despite guideline-concordant multimodal therapy: a case report.

Primary pulmonary synovial sarcoma (primary pulmonary synovial sarcoma) accounts for less than 0.5% of all primary lung malignancies.

It is characterized by an aggressive clinical course and a defining chromosomal translocation, t(X,18)(p11;q11), resulting in the fusion of the SYT gene on chromosome 18 with SSX1 or SSX2 on chromosome X.

A 60-year-old woman with a 50-pack-per-year smoking history presented with gross hematuria and was incidentally found to have a right lower lobe lung mass on chest radiography.

After four cycles, follow-up imaging showed tumor reduction greater than 30% and no evidence of metastatic disease.