In the last 30 years, bone and soft tissue tumors have seen the best period of development in history, especially the introduction of neoadjuvant and adjuvant chemotherapy, which has led to a significant improvement in the survival rate of patients with primary highly malignant bone tumors. Advances in surgical techniques have even led to higher patient satisfaction with overall treatment outcomes. At the same time, a plateau in survival rates for patients with osteosarcoma has emerged, making it difficult for new drugs and techniques to make further breakthroughs in this regard. This is the challenge that researchers in this field are currently working to overcome. For primary malignant bone tumors, the search for new targets for systemic therapy has been a focus of attention in recent years. In addition, attempts to identify prognostically relevant tumor markers are also part of the effort. In contrast, for benign tumors, the focus is on the progressive reduction of negative factors associated with therapeutic interventions over time. In the 2013 Chinese Society of Clinical Oncology (CSCO) Congress Bone Tumor Symposium, Prof. Xiaohui Niu, President of the Congress, reviewed the history and progress of the development of bone and soft tissue tumors, and the CSCO is committed to promoting standardized treatment and multidisciplinary collaboration, which has achieved initial results in national promotion. A certain consensus has been achieved in the treatment of primary tumors, especially the treatment pathway of classic osteosarcoma introduced in the last two CSCO annual meetings, which has been widely recognized by the industry. For osteosarcoma, the most common primary malignant tumor of bone, pathologic grade is critical in the staging. Patients with low-grade osteosarcoma do not require chemotherapy and their survival is virtually unaffected by the disease. In contrast, high-grade tumors are more likely to metastasize and are routinely treated with surgery combined with chemotherapy, with a survival rate of approximately 70%. We still need representative markers to predict the prognosis of patients with high-grade osteosarcoma. Exploration related to efficacy and prognosis prediction continues Histologic response to neoadjuvant chemotherapy remains the primary predictor of survival. Many methods have been used to predict chemotherapy response, including imaging morphology, circulating cytokines and genetic phenotype of tumor cells, and thus hopefully survival.Li et al. investigated the relationship between circulating chemokines and clinical regression in patients with osteosarcoma. The results showed that CXCL4 and CXCL6 were expressed in more than 90% of osteosarcoma cases, and the higher the expression level, the worse the patient’s prognosis. Newer studies have addressed some of the less common subtypes of highly malignant osteosarcoma. The 10-year survival rate for patients with periosteal osteosarcoma was reported to be 84%, and the overall survival (OS) rate for patients receiving chemotherapy and surgery was not significantly different from that of patients receiving surgery only.Ruggieri et al. confirmed the extremely poor prognosis for patients with pegylated osteosarcoma.Samartzis et al. included 80,181 survivors of the atomic bombing of Nagasaki, Hiroshima, in a prospective study and identified 19 cases of osteosarcoma arising in bone, with osteosarcoma being the most common cell type. The authors found a dose threshold of 0.85 Gy, well below the dose previously thought likely to induce secondary sarcoma. For all highly malignant osteosarcomas, surgery and chemotherapy remain the standard of care. Choy et al. identified multiple variants in the PI3K pathway in tissue-based osteosarcoma cell lines, and Yang et al. identified the vascular endothelial growth factor (VEGF) pathway (including VEGF-A) as a possible alternative target for this heterogenous malignancy. ciernik et al. investigated the effect of proton radiotherapy on local control and survival in 55 patients with unresectable or incompletely resected osteosarcoma . In this study, the mean total radiotherapy dose was 68.4 Gy, the 5-year OS rate was 67%, and the 5-year local control rate was 72%. Proton radiotherapy may provide good local control and survival benefit in selective osteosarcoma patients. Thallium uptake has been reconsidered as a predictor of event-free survival in patients with osteosarcoma. However, a follow-up study by Magnan et al. did not show a correlation between reduced thallium uptake and event-free survival. The authors suggested that thallium uptake should not be used as a predictor of necrosis.Ichikawa et al. studied the procoagulant features of osteosarcoma and found that the presence of osteosarcoma cells in small clots of venous emboli adjacent to the test tumor did correlate with poor prognosis. This is the reason why it is not appropriate to administer limb-preserving therapy when certain tumor cells invade the blood vessels. The 2013 NCCN bone tumor guidelines update the second-line treatment of osteosarcoma and add recommendations for the treatment of metastatic osteosarcoma, which is present in approximately 10% to 20% of patients with metastatic osteosarcoma at diagnosis and an event-free 2-year survival rate of 21%. The survival rate is 21% and the 2-year OS rate is 55%; the number of metastases and whether all lesions can be completely resected constitute independent prognostic factors. For the treatment of progressive osteosarcoma, preoperative chemotherapy followed by extensive resection of the primary tumor is recommended for cases with resectable metastases, and treatment of metastases includes chemotherapy and surgical resection. Research advances in different types of bone tumors Ewing sarcoma, a member of the Ewing family of tumors, differs from most primary tumors of bone in that it has a characteristic chromosomal translocation, most commonly between chromosomes 11 and 22. The protein product of this translocation is thought to be an abnormal transcription factor, which in turn drives the pathogenesis of Ewing sarcoma. ews-fli1 can form a transcriptional complex with RNA uncoupling enzyme A (RHA), and this complex is associated with the pathogenesis of Ewing sarcoma. Ewing sarcoma of bone origin is more likely to occur in younger patients, is more likely to be male and more often located in the extremities, and has a lower 5-year survival rate than Ewing sarcoma of soft tissue origin.Gupta et al. compared the survival rates of adult and pediatric patients with Ewing sarcoma. The authors found a 3-year OS rate of 81% in children compared to 59% in adults (p=0.02). They attributed some of this difference to the lower doses of chemotherapy drugs used in adults. In recent years, targeted therapies targeting the insulin-like growth factor (IGF) tyrosine kinase pathway have received a lot of attention. Zoledronic acid has been used to prevent adverse bone events in patients with metastatic bone cancer, and Odri et al. found that in a murine model implanted with Ewing sarcoma cells, zoledronic acid combined with one dose of isocyclophosphamide was as effective as three doses of isocyclophosphamide. The authors concluded that zoledronic acid can affect the conversion of osteoclasts and that combining it with low-dose conventional chemotherapeutic agents may reduce side effects while effectively treating tumors. Adherence to standardized and comprehensive treatment of tumors, multidisciplinary collaboration and multicenter clinical research have always been important driving factors for the development of our discipline. Chondrosarcoma Chondrosarcoma, as a common primary malignant tumor of bone, remains the best indicator of patient prognosis, and Chen et al. evaluated the role of the vonHippel-Lindau (VHL) tumor suppressor gene in chondrosarcoma and found that reduced VHL expression was associated with decreased apoptosis and higher histologic grade, but not with survival. The treatment of low-grade chondral lesions can still be of interest and remains controversial; Mohler et al. reported a 4.3% recurrence rate for the treatment of low-grade chondrosarcoma of the limb using curettage and cryotherapy and concluded that curettage and cryotherapy is a reasonable alternative to extensive resection for low-grade chondral lesions. Giant cell tumor of bone, the incidence of which is substantially higher in China than in Caucasians in Europe and the United States, occurs in the spine and sacrum, and recurrent giant cell tumors are a surgical challenge. Thomas et al. obtained positive results in a prospective trial using a monoclonal antibody to RANKL to target giant cell tumors. The premise of targeting RANKL is that receptors for RANKL are present on giant cells and that the activation of giant cells is partially associated with RANKL. If the activation of giant cells can be blocked by binding RANKL, the growth of giant cell tumors can be inhibited. Bisphosphonates have also been used for the local control of giant cell tumors. A recent study evaluated the cytotoxic effect of zoledronic acid on mesenchymal cells in giant cell tumors in vitro by eluting from acrylic bone cement. Available non-surgical treatment options include a series of arterial embolizations, denosumab, interferon or pegylated interferon. For metastases from giant cell tumors, intracapsular resection is an option for resectable metastatic lesions and non-surgical treatment options or radiation therapy for unresectable lesions. In the management of chordoma, the sacrum remains the most common site (50% – 60%) and the pathological types are classified as classic, chondrocyte-like and dedifferentiated. The incidence is extremely low and historically it is a difficult disease to treat, with a high recurrence rate. Surgery remains the only effective treatment. The use of radiotherapy in the treatment of chordoma remains acutely debated. Newer research reinforces the view that high-dose radiation therapy is effective in treating chordoma. Systemic treatment for this disease is still in the experimental stage. Extensive resection should be performed for the sacrum and movable vertebrae, and intracranial resection for the skull base. In cases with positive sarcomere margins or intracranial resection, postoperative radiotherapy is recommended to improve local control and to increase disease-free survival. In cases where surgical resection is not possible, radiotherapy is the primary treatment. For progressive chordoma, surgical treatment with adjuvant radiotherapy or drug therapy is effective. A study by the Rizzoli Bone Tumor Center in Italy showed a local recurrence rate of 17% for extensive resection and 81% for intracapsular or marginal resection. This shows that the recurrence rate is still very high. For resectable tumors, radiotherapy can be used as an adjuvant; for cases that cannot undergo surgery, radiotherapy can be used as a definitive final treatment. In conclusion, insistence on standardized and comprehensive treatment of tumors, multidisciplinary collaboration and multicenter clinical research are always important driving factors for the development of this discipline.