Highlights & Basics
- Osteosarcoma is the most common nonhematologic primary malignant neoplasm of bone in children and adolescents.
- Pain and swelling are the most common presenting symptoms.
- Although it can occur at any age, it usually affects patients in the second and third decade of life with a peak incidence between 13 and 16 years of age. It is more common in males than females.
- Chemotherapy combined with surgery is the standard of care.
- Prognosis of patients with localized disease has improved substantially following the introduction of chemotherapy, with 75% to 80% 5-year survival rates.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Conventional radiograph, anteroposterior view; poorly circumscribed, permeative lesion involving distal femoral metaphysis with mixed radiodense and radiolucent appearance; a large soft tissue mass with periosteal reaction is also present
Computed tomographic scan, axial view; osteosarcoma of proximal tibia; matrix production and bone destruction are best appreciated on conventional tomographs
Magnetic resonance imaging, coronal view; osteosarcoma of distal femur showing low-intensity signal; T1-weighted image; actual intraosseous and extraosseous tumor extent is also appreciated
Magnetic resonance imaging, axial view; osteosarcoma of distal femur showing high-intensity signal; T2-weighted image
Bone scan; high radionuclide uptake at tumor site
Osteoblastic osteosarcoma; lace-like osteoid in a highly pleomorphic sarcomatous stroma
Citations
WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours: WHO classification of tumours. 5th ed. vol 3. Lyon, France: IARD Press; 2020
Strauss SJ, Frezza AM, Abecassis N, et al. Bone sarcomas: ESMO-EURACAN-GENTURIS-ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2021 Dec;32(12):1520-36.[Abstract][Full Text]
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: bone cancer [internet publication].[Full Text]
1. WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours: WHO classification of tumours. 5th ed. vol 3. Lyon, France: IARD Press; 2020
2. Azar FM, Canale ST, Beaty JH. Campbell's operative orthopedics. 14th ed. Philadelphia, PA: Elsevier; 2021.
3. Mirabello L, Troisi RJ, Savage SA. Osteosarcoma incidence and survival rates from 1973 to 2004: data from the Surveillance, Epidemiology, and End Results Program. Cancer. 2009 Apr 1;115(7):1531-43.[Abstract][Full Text]
4. Ferguson WS, Goorin AM. Current treatment of osteosarcoma. Cancer Invest. 2001;19:292-315.[Abstract]
5. de Pinieux G, Karanian M, Le Loarer F, et al. Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network. PLoS One. 2021;16(2):e0246958.[Abstract][Full Text]
6. Gatta G, Capocaccia R, Botta L, et al. Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol. 2017 Aug;18(8):1022-39.[Abstract][Full Text]
7. Strauss SJ, Frezza AM, Abecassis N, et al. Bone sarcomas: ESMO-EURACAN-GENTURIS-ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2021 Dec;32(12):1520-36.[Abstract][Full Text]
8. Le Vu B, de Vathaire F, Shamsaldin A, et al. Radiation dose, chemotherapy and risk of osteosarcoma after solid tumours during childhood. Int J Cancer. 1998 Jul 29;77(3):370-7.[Abstract]
9. Campanacci M. Bone and soft tissue tumors: clinical features, imaging, pathology and treatment. 2nd ed. New York, NY: Springer; 1999.
10. Troisi R, Masters MN, Joshipura K, et al. Perinatal factors, growth and development, and osteosarcoma risk. Br J Cancer. 2006 Dec 4;95(11):1603-7.[Abstract][Full Text]
11. Martin JW, Squire JA, Zielenska M. The genetics of osteosarcoma. Sarcoma. 2012;2012:627254.[Abstract][Full Text]
12. Yamaguchi T, Toguchida J, Yamamuro T, et al. Allelotype analysis in osteosarcomas: frequent allele loss on 3q, 13q, 17p, and 18q. Cancer Res. 1992 May 1;52(9):2419-23.[Abstract][Full Text]
13. Overholtzer M, Rao PH, Favis R, et al. The presence of p53 mutations in human osteosarcomas correlates with high levels of genomic instability. Proc Natl Acad Sci U S A. 2003 Sep 30;100(20):11547-52.[Abstract][Full Text]
14. Miller CW, Aslo A, Won A, et al. Alterations of the p53, Rb and MDM2 genes in osteosarcoma. J Cancer Res Clin Oncol. 1996;122(9):559-65.[Abstract]
15. Hansen MF, Nellissery MJ, Bhatia P. Common mechanisms of osteosarcoma and Paget's disease. J Bone Miner Res. 1999 Oct;14 Suppl 2:39-44.[Abstract]
16. Wang LL, Gannavarapu A, Kozinetz CA, et al. Association between osteosarcoma and deleterious mutations in the RECQL4 gene in Rothmund-Thomson syndrome. J Natl Cancer Inst. 2003 May 7;95(9):669-74.[Abstract]
17. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: bone cancer [internet publication].[Full Text]
18. Azar FM, Canale ST, Beaty JH. Campbell's operative orthopedics. 14th ed. Philadelphia, PA: Elsevier; 2021.
19. Campanacci M. Bone and soft tissue tumors: clinical features, imaging, pathology and treatment. 2nd ed. New York, NY: Springer; 1999.
20. Letson D, Falcone R, Muro-Cacho C. Pathologic and radiologic features of primary bone tumors. Cancer Control. 1999 May;6(3):283-93.[Abstract]
21. Malawer MM, Sugarbaker PH. Musculoskeletal cancer surgery: treatment of sarcomas and allied diseases. Dordrecht: Kluwer Academic Publishers; 2001.[Full Text]
22. Meyer JS, Nadel HR, Marina N, et al. Imaging guidelines for children with Ewing sarcoma and osteosarcoma: a report from the Children's Oncology Group Bone Tumor Committee. Pediatr Blood Cancer. 2008 Aug;51(2):163-70.[Abstract]
23. Letson D, Falcone R, Muro-Cacho C. Pathologic and radiologic features of primary bone tumors. Cancer Control. 1999;6:283-293.[Abstract]
24. Heck RK Jr, Peabody TD, Simon MA. Staging of primary malignancies of bone. CA Cancer J Clin. 2006 Nov-Dec;56(6):366-75.[Abstract][Full Text]
25. Amin MB, Edge S, Greene F, et al. AJCC cancer staging manual, 8th edition. New York: Springer; 2016.
26. American College of Radiology. ACR appropriateness criteria: malignant or aggressive primary musculoskeletal tumor-staging and surveillance. 2022 [internet publication].[Full Text]
27. Ferrari S, Ruggieri P, Cefalo G, et al. Neoadjuvant chemotherapy with methotrexate, cisplatin, and doxorubicin with or without ifosfamide in nonmetastatic osteosarcoma of the extremity: an Italian sarcoma group trial ISG/OS-1. J Clin Oncol. 2012 Jun 10;30(17):2112-8.[Abstract]
28. Marina NM, Smeland S, Bielack SS, et al. Comparison of MAPIE versus MAP in patients with a poor response to preoperative chemotherapy for newly diagnosed high-grade osteosarcoma (EURAMOS-1): an open-label, international, randomised controlled trial. Lancet Oncol. 2016 Oct;17(10):1396-408.[Abstract][Full Text]
29. Anninga JK, Gelderblom H, Fiocco M, et al. Chemotherapeutic adjuvant treatment for osteosarcoma: where do we stand? Eur J Cancer. 2011 Nov;47(16):2431-45.[Abstract]
30. Beird HC, Bielack SS, Flanagan AM, et al. Osteosarcoma. Nat Rev Dis Primers. 2022 Dec 8;8(1):77.[Abstract]
31. Malawer MM, Sugarbaker PH. Musculoskeletal cancer surgery: treatment of sarcomas and allied diseases. Dordrecht: Kluwer Academic Publishers; 2001.
32. Goorin AM, Schwartzentruber DJ, Devidas M, et al. Presurgical chemotherapy compared with immediate surgery and adjuvant chemotherapy for nonmetastatic osteosarcoma. J Clin Oncol. 2003 Apr 15;21(8):1574-80.[Abstract]
33. Bacci G, Mercuri M, Longhi A, et al. Grade of chemotherapy-induced necrosis as a predictor factor of local and systemic control in 881 patients with non-metastatic osteosarcoma of the extremities treated with neoadjuvant chemotherapy in a single institution. Eur J Cancer. 2005 Sep;41(14):2079-85.[Abstract]
34. Rosen G, Marcove RC, Huvos AG, et al. Primary osteogenic sarcoma: eight-year experience with adjuvant chemotherapy. J Cancer Res Clin Oncol. 1983;106 Suppl:55-67.[Abstract]
35. Bielack SS, Kempf-Bielack B, Delling G, et al. Prognostic factors in high-grade osteosarcoma of the extremities and trunk: an analysis of 1,702 patients treated on neoadjuvant cooperative osteosarcoma study group protocols. J Clin Oncol. 2002 Feb 1;20(3):776-90.[Abstract]
36. Meyers PA, Heller G, Healey J, et al. Chemotherapy for nonmetastatic osteogenic sarcoma: the Memorial Sloan-Kettering experience. J Clin Oncol. 1992 Jan;10(1):5-15.[Abstract]
37. Anderson ME. Update on survival in osteosarcoma. Orthop Clin North Am. 2016 Jan;47(1):283-92.[Abstract]
38. Kager L, Zoubek A, Kastner U, et al. Primary metastatic osteosarcoma: presentation and outcome of patients treated on neoadjuvant Cooperative Osteosarcoma Study Group protocols. J Clin Oncol. 2003 May 15;21(10):2011-8.[Abstract]
39. Davis LE, Bolejack V, Ryan CW, et al. Randomized double-blind phase II study of regorafenib in patients with metastatic osteosarcoma. J Clin Oncol. 2019 Jun 1;37(16):1424-31.[Abstract][Full Text]
40. American College of Rheumatology. 2022 American College of Rheumatology (ACR) guideline for vaccinations in patients with rheumatic and musculoskeletal diseases. Feb 2023 [internet publication].[Full Text]
41. Haanen J, Obeid M, Spain L, et al. Management of toxicities from immunotherapy: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2022 Dec;33(12):1217-38.[Abstract][Full Text]
42. Hendershot E, Pappo A, Malkin D, et al. Tumor necrosis in pediatric osteosarcoma: impact of modern therapies. J Pediatr Oncol Nurs. 2006 Jul-Aug;23(4):176-81.[Abstract]
