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.