Bone Cancer: Diagnosis and Treatment Principles

 

Am Fam Physician. 2018 Aug 15;98(4):205-213.

  Patient information: See related handout on bone cancer, written by the authors of this article.

Author disclosure: No relevant financial affiliations.

Primary bone cancers include osteosarcoma, Ewing sarcoma, and chondrosarcoma. They account for less than 1% of diagnosed cancers each year and are associated with significant morbidity and mortality. Timely diagnosis is challenging because of late patient presentation, nonspecific symptoms that mimic common musculoskeletal injuries, and low suspicion by physicians. Plain radiography is the preferred diagnostic test. Radiographic suspicion of a bone malignancy should prompt quick referral to a cancer center for multidisciplinary care. Osteosarcoma, the most common bone cancer, most often occurs in children and adolescents. It typically develops in the metaphysis of long bones, specifically the distal femur, proximal tibia, and proximal humerus. Metastasis to the lungs is common. Use of neoadjuvant and adjuvant chemotherapy, in combination with surgery, has improved survival rates to nearly 80% for patients with localized disease, and 90% to 95% of patients do not require limb amputation. Ewing sarcoma is the second most common bone cancer and is similar to osteosarcoma in terms of presenting symptoms, age at occurrence, and treatment. Prognosis for osteosarcoma and Ewing sarcoma depends on the presence of metastasis, which lowers the five-year survival rate to 20% to 30%. Chondrosarcoma is the rarest bone cancer, primarily affecting adults older than 40 years. Survival rates are higher because most of these tumors are low-grade lesions.

Skeletal bones can host numerous types of cancers. They commonly receive metastases from other malignancies, including breast, lung, renal, prostate, and thyroid cancers. Bone marrow can be the nidus of malignancy in multiple myeloma, lymphoma, and leukemia. In all of these illnesses, however, the malignant cells are not of bony origin.

 Enlarge     Print

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Bony pain that manifests after minor trauma, persists, causes nighttime awakening, or is associated with swelling should be evaluated with radiography.

C

2, 3, 10, 11

Patients with suspected bone cancer should be referred immediately to a cancer center experienced with malignant bone tumors.

C

14, 15

Biopsy of a suspected bone cancer should be performed by an experienced surgeon.

C

5, 14


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Bony pain that manifests after minor trauma, persists, causes nighttime awakening, or is associated with swelling should be evaluated with radiography.

C

2, 3, 10, 11

Patients with suspected bone cancer should be referred immediately to a cancer center experienced with malignant bone tumors.

C

14, 15

Biopsy of a suspected bone cancer should be performed by an experienced surgeon.

C

5, 14


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

Three types of cancers arise in bone itself: osteosarcoma, Ewing sarcoma, and chondrosarcoma. Although these malignancies account for less than 1% of all diagnosed cancers each year, their morbidity and mortality are significant.1 This article reviews the characteristics of primary bone cancers, their diagnosis, treatment, and prognoses.

Osteosarcoma

Osteosarcoma is the most common bone cancer, accounting for nearly two-thirds of all cases.24 Approximately 1,200 patients are diagnosed with osteosarcoma in the United States annually.1 Osteosarcoma is primarily an illness of childhood, with a small increase in incidence among persons older than 60 years. It is the third most common childhood malignancy, with a median incidence at 12 years of age for girls and 16 years for boys.2

Osteosarcoma is thought to originate from malignant primitive mesenchymal cells that differentiate into osteoblasts, which in turn produce a malignant osteoid matrix. Osteosarcomas can arise in any bone, but classically develop in the metaphyses of long bones. Nearly 60% occur in the distal femur, the proximal tibia, and the proximal humerus.3 A bone’s metaphysis contains the growth plate, which is responsible for active bone formation and elongation. Thus, osteosarcomas tend to occur at the age and

The Authors

show all author info

JASON L. FERGUSON, DO, MBA, is director of the Tripler Army Family Medicine Residency Program, Honolulu, Hawaii, and assistant professor of family medicine at the Uniformed Services University of the Health Sciences, Bethesda, Md....

SEAN P. TURNER, MD, is a family physician at Schofield Barracks, Honolulu, Hawaii.

Address correspondence to Jason L. Ferguson, DO, Tripler Army Medical Center, 1 Jarrett White Rd., Honolulu, HI 96859 (e-mail: jason.l.ferguson.mil@mail.mil). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

References

show all references

1. American Cancer Society. Cancer facts & figures 2008. https://www.cancer.org/research/cancer-facts-statistics/all-cancer-facts-figures/cancer-facts-figures-2008.html. Accessed May 30, 2018....

2. Lieberman JR. Malignant bone tumors. In: AAOS Comprehensive Orthopaedic Review. Rosemont, Ill.: American Academy of Orthopaedic Surgeons; 2009:417–442.

3. Marina N. Malignant bone tumors. In: Lanzkowsky P, ed. Manual of Pediatric Hematology and Oncology. 5th ed. London, UK: Academic Press; 2010:739–757.

4. National Cancer Institute. Ewing sarcoma treatment (PDQ)—health professional version. April 4, 2018. https://www.cancer.gov/types/bone/hp/ewing-treatment-pdq. Accessed May 30, 2018.

5. Wittig JC, Bickels J, Priebat D, et al. Osteosarcoma: a multidisciplinary approach to diagnosis and treatment. Am Fam Physician. 2002;65(6):1123–1132.

6. Rajwanshi A, Srinivas R, Upasana G. Malignant small round cell tumors. J Cytol. 2009;26(1):1–10.

7. Giuffrida AY, Burgueno JE, Koniaris LG, Gutierrez JC, Duncan R, Scully SP. Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database. J Bone Joint Surg Am. 2009;91(5):1063–1072.

8. Wold LE. Osteosarcoma. In: Atlas of Orthopedic Pathology. 3rd ed. Philadelphia, Pa.: Saunders/Elsevier; 2008:211–212, 297–298, 427–428.

9. Miller RW, Boice JD Jr., Curtis RE. Bone cancer. In: Schottenfeld D, Fraumeni JF Jr., eds. Cancer Epidemiology and Prevention. 3rd ed. New York: Oxford University Press; 2006:946–958.

10. Pan KL, Chan WH, Chia YY. Initial symptoms and delayed diagnosis of osteosarcoma around the knee joint. J Orthop Surg (Hong Kong). 2010;18(1):55–57.

11. Widhe B, Widhe T. Initial symptoms and clinical features in osteosarcoma and Ewing sarcoma. J Bone Joint Surg Am. 2000;82(5):667–674.

12. Jawad MU, Cheung MC, Min ES, Schneiderbauer MM, Koniaris LG, Scully SP. Ewing sarcoma demonstrates racial disparities in incidence-related and sex-related differences in outcome: an analysis of 1631 cases from the SEER database, 1973–2005. Cancer. 2009;115(15):3526–3536.

13. American College of Radiology. ACR Appropriateness Criteria: primary bone tumors. https://acsearch.acr.org/docs/69421/Narrative/. Accessed October 20, 2017.

14. National Collaborating Centre for Cancer; National Institute for Health and Clinical Excellence . Improving Outcomes for People with Sarcoma: The Manual. London, UK: National Institute for Health and Clinical Excellence; 2006:1–142.

15. National Cancer Institute. Osteosarcoma and malignant fibrous histiocytoma of bone treatment (PDQ): health professional version. November 13, 2017. https://www.cancer.gov/types/bone/hp/osteosarcoma-treatment-pdq. Accessed May 30, 2018.

16. Anderson ME. Update on survival in osteosarcoma. Orthop Clin North Am. 2016;47(1):283–292.

17. Bernthal NM, Federman N, Eilber FR, et al. Long-term results (>25 years) of a randomized, prospective clinical trial evaluating chemotherapy in patients with high-grade, operable osteosarcoma. Cancer. 2012;118(23):5888–5893.

18. Bielack SS, Hecker-Nolting S, Blattmann C, Kager L. Advances in the management of osteosarcoma. F1000Res. 2016;5:2767.

19. Jones KB, Ferguson PC, Lam B, et al. Effects of neoadjuvant chemotherapy on image-directed planning of surgical resection for distal femoral osteosarcoma. J Bone Joint Surg Am. 2012;94(15):1399–1405.

20. Reed DR, Hayashi M, Wagner L, et al. Treatment pathway of bone sarcoma in children, adolescents, and young adults. Cancer. 2017;123(12):2206–2218.

21. Bielack SS, Kempf-Bielack B, Delling G, et al. Prognostic factors in high-grade osteosarcoma of the extremities or trunk: an analysis of 1,702 patients treated on neoadjuvant cooperative osteosarcoma study group protocols. J Clin Oncol. 2002;20(3):776–790.

22. Biswas B, Rastogi S, Khan SA, et al. Outcomes and prognostic factors for Ewing-family tumors of the extremities. J Bone Joint Surg Am. 2014;96(10):841–849.

23. Duchman KR, Gao Y, Miller BJ. Prognostic factors for survival in patients with high-grade osteosarcoma using the Surveillance, Epidemiology, and End Results (SEER) Program database. Cancer Epidemiol. 2015;39(4):593–599.

 

 

Copyright © 2018 by the American Academy of Family Physicians.
This content is owned by the AAFP. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. Contact afpserv@aafp.org for copyright questions and/or permission requests.

Want to use this article elsewhere? Get Permissions

CME Quiz

More in AFP


Editor's Collections


Related Content


More in Pubmed

MOST RECENT ISSUE


Nov 15, 2018

Access the latest issue of American Family Physician

Read the Issue


Email Alerts

Don't miss a single issue. Sign up for the free AFP email table of contents.

Sign Up Now

Navigate this Article