RESUMO
Osteoid osteoma and osteoblastoma are the most important benign osteoid-forming tumors. They grow slowly and are well differentiated. Histologically, the tumor cells show no atypia and no increased mitoses. In typical cases, they can be clearly diagnosed. However, the rare cases on the dividing line between osteoblastoma and osteosarcoma are extremely problematic. In these cases, molecular genetic investigations should contribute to finding the correct diagnosis in the future.Juvenile highly malignant osteosarcoma is the most important malignant osteoid-forming tumor. About 40 years ago, neoadjuvant chemotherapy was introduced for the mostly young patients. This therapy highly significantly improved prognosis. However, a plateau phase was quickly reached and the last several decades have seen no further progress in conventional therapeutic approaches. There is no doubt that further progress can only be achieved on the basis of new molecular genetic and cell biological findings. The target-therapeutic strategies derived from these findings will be discussed in this review.The rare parosteal osteosarcoma and the even rarer periosteal osteosarcoma are mostly not highly malignant tumors that are located on the surface of bone. The parosteal osteosarcoma is usually G1 and the periosteal osteosarcoma G2. Occasionally, the differential diagnosis between a parosteal osteosarcoma and a fibrous dysplasia is difficult. In such rare cases, the detection of GNAS mutations in fibrous dysplasia can prove useful. In contrast to chondromas and chondrosarcomas, periosteal osteosarcomas do not contain IDH1 and IDH2 mutations.
