Primary rhabdomyosarcoma of the distal femoral diaphysis: a case report and review of the literature.

Primary rhabdomyosarcoma of the bone is an extremely rare condition with few examples reported in the literature. We present the case of a 34-year-old male who presented with a lesion in the distal femur with initial imaging features consistent with Ewing sarcoma. Histologically, the lesion consisted of atypical pleomorphic polygonal rhabdomyoblasts demonstrating focal desmin and myogenin expression. A diagnosis of pleomorphic rhabdomyosarcoma was rendered. Despite systemic treatment and surgery, this patient experienced a rapidly progressive disease course. We believe this is only the second report in the orthopedic literature of a case of primary pleomorphic rhabdomyosarcoma of the bone. The key imaging, pathologic, and clinical findings are discussed.