Introduction:
Primary bone composite sarcoma is a rare entity that accounts for less than 0.5% of bone sarcomas. It is diagnosed during adolescence and young adulthood, and usually combines areas of liposarcoma with osteosarcoma. The presence of disseminated pulmonary disease occurs in up to 60% of patients and, with conventional treatment schemes for osteosarcoma, survival rates do not exceed 30% (1-5).
We present the case of an adolescent female diagnosed with a primary agressive bone composite sarcoma with rhabdomyosarcoma foci and loco-regional lymph node involvement.