期刊
NATURE REVIEWS DISEASE PRIMERS
卷 8, 期 1, 页码 -出版社
NATURE PORTFOLIO
DOI: 10.1038/s41572-022-00409-y
关键词
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资金
- Foster Foundation
- Swim Across America Inc
- Osteosarcoma Institute
- QuadW Foundation
- Barbara Epstein Foundation
- Tom Prince Cancer Trust
- Bone Cancer Research Trust
- Sarcoma UK
- Cancer Research UK University College London Experimental Cancer Medicine Centre
- RNOH Research and Development Department
- National Institute for Health Research
- University College London Hospitals Biomedical Research Centre
- Triumph Over Kid Cancer Foundation
- A Shelter for Cancer Families
- QuadW Foundation
- Cancer Prevention Research Institute of Texa
- Rally Foundation
- Make It Better (MIB) Agents
- National Institutes of Health/National Cancer Institute
- CancerFREE Kids Foundation
- Steps for Sarcoma
- St. Baldrick's Foundation
- ICO Cancer Center, France
- Ouest Valorisation SATT (France) [DorSarc-2018-ICO-DH]
- Bone Cancer Research Trust (UK)
- Pan Mass Challenge and philanthropic funds
- osteosarcoma research at Dana-Farber Cancer Institute
- Foerderkreis krebskranke Kinder Stuttgart e.V
Osteosarcoma is the most common primary malignant bone tumor with a bimodal incidence peaking at 18 and 60 years of age. It is more common in males and is driven by genetic factors related to bone formation leading to malignant progression and metastasis. Screening is currently focused on high-risk groups, and the prognosis for patients with metastatic disease remains poor.
Osteosarcoma is the most common primary malignant tumour of the bone. Osteosarcoma incidence is bimodal, peaking at 18 and 60 years of age, and is slightly more common in males. The key pathophysiological mechanism involves several possible genetic drivers of disease linked to bone formation, causing malignant progression and metastasis. While there have been significant improvements in the outcome of patients with localized disease, with event-free survival outcomes exceeding 60%, in patients with metastatic disease, event-free survival outcomes remain poor at less than 30%. The suspicion of osteosarcoma based on radiographs still requires pathological evaluation of a bone biopsy specimen for definitive diagnosis and CT imaging of the chest should be performed to identify lung nodules. So far, population-based screening and surveillance strategies have not been implemented due to the rarity of osteosarcoma and the lack of reliable markers. Current screening focuses only on groups at high risk such as patients with genetic cancer predisposition syndromes. Management of osteosarcoma requires a multidisciplinary team of paediatric and medical oncologists, orthopaedic and general surgeons, pathologists, radiologists and specialist nurses. Survivors of osteosarcoma require specialized medical follow-up, as curative treatment consisting of chemotherapy and surgery has long-term adverse effects, which also affect the quality of life of patients. The development of osteosarcoma model systems and related research as well as the evaluation of new treatment approaches are ongoing to improve disease outcomes, especially for patients with metastases.
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