Abstract
Primary intraosseous poorly differentiated synovial sarcoma is exceedingly rare. Here, we present a case of primary intraosseous poorly differentiated synovial sarcoma from the proximal femur in a 16-year-old girl. The case was initially misdiagnosed, but the correct diagnosis of synovial sarcoma was eventually confirmed by fluorescence in situ hybridization and next-generation sequencing. We review the literature pertaining to synovial sarcoma and show that this case is the second molecularly proven intraosseous poorly differentiated synovial sarcoma in the literature. Recognition of intraosseous synovial sarcoma composed of small round cells is imperative in order to avoid misdiagnosis of the tumor as Ewing sarcoma and other small round-cell tumors, all of which have markedly different clinical management.