All About Sarcomas of the Bone

Carolyn Vachani, RN, MSN, AOCN
Updated by Christina Bach, MBE, MSW, LCSW, OSW-C
The Abramson Cancer Center of the University of Pennsylvania
Last Modified: May 20, 2016

What is sarcoma?

Sarcoma is a cancer of the soft tissue (muscle, fat, nerve, or connective tissue) or bone. (This article will discuss sarcomas of the bone; soft tissue sarcomas are discussed in a separate article). Sarcomas encompass a group of over 40 different types of tumors. In general, sarcomas are considered quite rare, accounting for less than 1% of all adult cancer diagnoses, with approximately 10,000 new cases annually in the soft tissues and 3,300 new cases annually in the bone. Sarcomas are considered primary bone cancers, which are different from bone metastases that have spread from cancer in other areas of the body. Sarcomas are named based on the site from which they arise. For example, chondrosarcomas arise from cartilage, osteosarcomas arise from bone, and fibrosarcomas arise from fibrogenic tissue. Determining the type of sarcoma is critical to appropriate treatment selection.

In adults, chondrosarcoma is the most common type of bone sarcoma, accounting for approximately 40% of bone tumors. Osteosarcoma is the second most common type in adults (accounting for 28% of bone sarcomas). In children and adolescents, ostersarcoma and Ewing's are the most common types of sarcoma. All other types are extremely rare, each accounting for less than 1% of all bone sarcomas. (See list below for some types of sarcomas of the bone). Within these types, there are further subtypes -- for example, there are 11 different types of osteosarcomas.

Ewing’s sarcoma can occur in the bone or soft tissue (called extra-osseous). This differentiation is important when determining treatment options. The large majority of cases occur in the bones, and the diagnosis is most common in the teenage years. PNET (primitive neuroectodermal tumor) is a type of sarcoma closely related to Ewing’s. PNET can occur in the bone or the central nervous system. PNET of the bone is treated the same as Ewing’s sarcoma, but PNET in the brain or spinal cord is a genetically different tumor, that is treated differently. Ewing’s, PNET, Askin’s tumors and neuroepithelioma all contain the same genetic abnormality, called an 11-22 chromosomal translocation (t11;22). Because of this similarity, these tumors are referred to as the Ewing’s sarcoma family of tumors and are treated similarly.

Am I at risk for sarcoma?

The cause of sarcoma of the bone (SB) is unknown in the large majority of cases. SB can develop from benign lesions in the bone or in areas that previously received radiation (these cases are rare and tend to occur many years after the radiation). In addition, a familial genetic syndrome called Li Fraumeni and a disease called Paget's, are associated with sarcomas. For the most part, doctors do not know why SBs occur and therefore cannot determine who is at risk of developing the disease.

The rates of osteosarcoma and Ewing’s sarcoma are the same for boys and girls until 13 years of age, after which males are more commonly affected. Ewing’s is 10 times more common in Caucasians then in blacks (either American or African) or Asians.

What screening tests are available?

Unfortunately, there is no screening test for SB. Screening tests are developed for the early detection of common or very deadly diseases. Given how rare SBs are, they would be difficult to screen for in the general population. In addition, the number of different types of SB would make it very difficult to develop one single screening test that could detect all types.

What are the signs of sarcoma of the bone?

The primary sign of SB is pain, with or without a mass that can be felt. The area of pain depends on the area involved with tumor. SBs occur most commonly in the long bones (thigh or femur, upper arm or humerus) or the pelvis. Pelvic tumors may not cause symptoms until they are larger, or if they do cause symptoms, they may not be correctly diagnosed right away because of the rare nature of SB.

How are sarcomas of the bone diagnosed?

Given how rare sarcomas are, many physicians have never seen or cared for a patient with sarcoma. When sarcoma is suspected, it is important to seek out a physician team familiar with sarcoma.

X-rays are the most useful initial radiology study to evaluate the tumor. A bone scan may be performed to evaluate the entire skeleton for other lesions. CT scan of the chest is usually done to rule out any metastases (spread) to the lungs (this is the most common site of spread). MRI (magnetic resonance imaging) with or without CT scan will be done in preparation for surgery. In some cases, PET scan may be used to detect any tumor metastases.

A biopsy is critical for diagnosis and to determine the exact type of sarcoma. Successful biopsy requires knowledge of sarcomas and their treatment, and is best done by a surgeon familiar with sarcoma, followed by examination of the sample by a pathologist who has experience with sarcoma specimens. Biopsies can be performed as an open (surgical) procedure or a closed (percutaneous) procedure (using a large needle to remove the tissue). The biopsy must be performed properly to collect enough tissue to get a diagnosis, but not so much tissue that it would compromise the definitive surgical treatment of the tumor. In general, the preferred method is the least invasive technique that still allows the pathologist to give a definitive diagnosis.

How are sarcomas of the bone staged?

Most tumor types are staged using a system developed by experts, but this has been difficult to develop for STS given the number of types and the varying locations. The most widely used system has been developed by the American Joint Committee on Cancer (AJCC) (7th edition). It incorporates tumor size, histologic grade (how different the cells look under the microscope when compared to normal cells), and spread to lymph nodes or other body sites in determining the stage. The "T stage" represents the extent of the primary tumor itself. The "N stage" represents the degree of involvement of the lymph nodes. The "M stage" represents whether or not there is spread of the cancer to distant parts of the body. In sarcomas, a further classification, the “G,” or histologic grade is also taken into account in staging. Histology refers to the appearance of the tumor cells under the microscope. The T, N, G, and M are combined to assign a stage, from I (one) denoting more limited disease, to IV (four) denoting more advanced disease.

AJCC Staging System for Soft Tissue Sarcomas (7th Edition 2010)


Primary Tumor (T)

Tx

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Tumor 8cm or less in greatest dimension

T2

Tumor more than 8cm in greatest dimension

T3

Discontinuous tumors in the primary bone site

Regional Lymph Nodes (N)

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Regional lymph node metastasis

Note: Because of the rarity of lymph node involvement in bone sarcomas, the designation NX may not be appropriate and cases should be considered N0 unless clinical node involvement is clearly evident.

Distant Metastasis (M)

M0

No distant metastasis

M1

Distant metastasis

M1a

Lung metastasis

M1b

Metastasis to other distant sites

Histologic Grade (G)

GX

Grade cannot be assessed

G1

Well differentiated-low grade

G2

Moderately differentiated-low grade

G3

Poorly differentiated

G4

Undifferentiated

Note: Ewing’s sarcoma is classified as G4.

Stage Grouping

Stage 1A

T1

N0

M0

G1,2(low grade), GX

Stage 1B

T2

N0

M0

G1, 2(low grade), GX

T3

N0

M0

G1, 2 (low grade), GX

Stage 2A

T1

N0

M0

G3, 4 (high grade)

Stage 2B

T2

M0

M0

G3, 4 (high grade)

Stage 3

T3

N0

M0

G3

Stage 4A

Any T

N0

M1a

Any G

Stage 4B

Any T

N1

Any M

Any G

Any T

Any N

M1B

Any G

In addition, the Surgical Staging System (SSS) may also be used in the staging of sarcomas of the bone. This system was developed by the Musculoskeletal Tumor Society and takes into account the surgical grade (G), local extent (T) and the absence or presence of regional or distant metastases.

Stage

Grade

Site

1A

Low (G1)

Intracompartmental (T1)

1B

Low (G1)

Extracompartmental (T2)

2A

High (G2)

Intracompartmental (T1)

2b

High (G2)

Extracompartmental (T2)

3

Any (G) + regional or distant mestastasis

Any (T)

How is sarcoma of the bone treated?

Given the rarity of SB, these patients are best served at a specialty treatment center. A Swedish study demonstrated that local recurrence rates were 2 and a half times higher in patients not referred to specialty centers. The study also found worse outcomes in patients who were referred to specialty centers after their initial surgery. Treatment of SB requires complex multimodality therapy (surgery, radiation and chemotherapy). Patients with suspected sarcomas should be referred to an orthopedic oncologist for biopsy and diagnosis.

Specific treatment is dependent upon the size and location of the tumor, the grade (aggressiveness) of the tumor, and whether or not it has spread. The following is a general review of current treatments, but specific cases should be discussed with the doctors on the team.

Surgery

Surgery is the primary means of treatment in SB. The goal is complete tumor removal. In patients with tumors in the arms or legs, this historically has meant amputation. With advances in orthopedic oncology, over 90% of patients with extremity tumors are now having limb-sparing surgery. In addition to the tumor, the surgeon typically removes a 2-cm area of normal tissue around the tumor whenever possible (to obtain "clear wide margins"). There is a low risk of spread to lymph nodes, therefore lymph node dissection is not routinely performed.

SBs that metastasize generally spread to the lungs first. Researchers have learned that by surgically removing metastases limited to the lungs, they can greatly improve survival in what would otherwise be a dismal prognosis. This is not a small procedure, so patients have to be healthy enough to endure a surgical resection of the lung tumor(s).

Radiation Therapy

Radiation therapy can be performed before or after surgery, or even during surgery through the use of brachytherapy. Radiation therapy can be used to treat tumors when they are not resectable with surgery, when clear margins are not achieved with surgery, or when there is disease recurrence (at the site of the original tumor or other localized site). Radiation is used in the treatment of chondrosarcomas more often than other types of SBs.

Chemotherapy

Unlike soft tissue sarcomas, doctors have had success in treating bone sarcomas with chemotherapy. Chemotherapy can be given before surgery in order to shrink the tumor and allow for a better resection, or it can be given after surgery. Surgery and radiation can only act on a small area around the tumor site, whereas the main goal behind chemotherapy is to kill any cancer cells floating undetected elsewhere in the body. It is these cells that can plant themselves and start to grow in other organs, most commonly the lungs.

Of the available chemotherapies, different ones work better in the different types of SBs. For example, in Ewing's sarcoma, ifosfamide, cyclophosphamide, etoposide, doxorubicin and vincristine are the most effective medications. In osteosarcoma, doxorubicin, cisplatin, carboplatin, ifosfamide and methotrexate are more effective. These medications are generally used in combinations of several drugs that work in different ways.

Chordomas are often treated with targeted therapies called tyrosine kinase inhibitors or multikinase inhibitors. Tyrosine kinase inhibitors are designed to block the action of a specific enzyme called tyrosine kinase. This enzyme plays a big role in the function of cells, and is active in cancer cells to promote tumor growth and progression. Some of these medications used in the treatment of chordomas are lapatinib, erlotinib, imatinib, and sunitinib.

Clinical Trials

Clinical trials are extremely important in furthering our knowledge of this disease. It is though clinical trials that we know what we do today, and many exciting new therapies are currently being tested. Talk to your healthcare provider about participating in clinical trials in your area.

Follow-Up Care and Survivorship

Follow-up care varies depending on the type and grade of the tumor, yet another reason to have these patients managed at specialty centers. Below are some guidelines put forth by the National Comprehensive Cancer Network on various SBs.

Osteosarcoma

Follow-up should include physical exam, chest x-ray and x-ray of the primary tumor site. This should be performed every 3 months for 2 years, then every 4 months for 1 year, then every 6 months for 2 years, then annually.

Chondrosarcoma

Follow-up should consist of physical exam, chest x-ray, and x-ray of primary tumor site. For low grade tumors: every 6 months for 2 years, then annually. For high grade tumors: every 3-6 months for 5 years, then annually for a minimum of 10 years.

Ewing's Sarcoma

Follow-up should include physical exam and chest x-ray. Every 2-3 months for 2 years, gradually increase the time span between visits, and switch to annual visits after 5 years. MRI of the primary tumor site should also be performed every 6 months for 2 years.

Other Concerns in Survivorship

Fear of recurrence, relationship challenges, financial impact of cancer treatment, employment issues and coping strategies are common emotional and practical issues experienced by sarcoma of the bone survivors. Your healthcare team can identify resources for support and management of these practical and emotional challenges faced during and after cancer. 

Cancer survivorship is a relatively new focus of oncology care. With some 15 million cancer survivors in the US alone, there is a need to help patients transition from active treatment to survivorship. What happens next, how do you get back to normal, what should you know and do to live healthy going forward? A survivorship care plan can be a first step in educating yourself about navigating life after cancer and helping you communicate knowledgeably with your healthcare providers. Create a survivorship care plan today on OncoLink.

Types of Sarcoma of the Bone

(This list does not contain all types)

  • Adamantinoma
  • Angiosarcoma
  • Chondrosarcoma
  • Chordoma
  • Clear Cell Chondrosarcoma
  • Classic Osteosarcoma
  • Ewing 's sarcoma of bone
  • Fibrosarcoma
  • Giant cell tumor
  • Hemangiopericytoma
  • High-Grade Surface Osteosarcoma
  • Intraosseous Osteosarcoma
  • Malignant fibrous histiocytoma
  • Mesenchymal Chondrosarcoma
  • Neurofibroma of bone (schwannoma)
  • Osteoblastoma
  • Osteochondroma
  • Osteosarcoma
  • Paget's disease / Pagetoid osteosarcoma
  • Periosteal Osteosarcoma
  • Pathological Fracture Risk assessment
  • Periosteal Chondroma
  • Periosteal Osteosarcoma
  • Peripheral neuroectodermal tumor (PNET)
  • Primitive neuroectodermal tumor of bone
  • Small Cell Osteosarcoma
  • Telangiectatic Osteosarcoma

Resources for More Information

Sarcoma Foundation of America
The SFA raises money for sarcoma research and aims to raise awareness of sarcoma. The site has information for patients as well.
http://www.curesarcoma.org/

Sarcoma Alliance
This website, started by a sarcoma survivor, is based on the mantra "guidance, education, and support". They also maintain a list of specialty centers.
http://www.sarcomaalliance.org

Bone Tumor.org
A site maintained by Dr. Henry DeGroot, an orthopedic oncologist from the University of Massachusetts Medical School. It provides information on a comprehensive list of types of bone tumors in English and Spanish.
http://www.bonetumor.org/

Amschwand Sarcoma Cancer Foundation
Aims to educate the public about sarcoma, support those with the disease, and encourage sarcoma-specific research. Provides a list of sarcoma treatment centers and housing information. http://www.sarcomacancer.org/

Chordoma Foundation
A nonprofit organization working to improve the lives of chordoma patients by accelerating research to develop effective treatments for chordoma, and by helping patients to get the best care possible.
http://www.chordomafoundation.org/

References

SEER Statistics: Bone Cancers http://seer.cancer.gov/statfacts/html/bones.html

NCCN Guidelines (registration required): www.nccn.org

Burningham, Z., Hashibe, M., Spector, L., & Schiffman, J. D. (2012). The epidemiology of sarcoma. Clinical Sarcoma Research, 2(1), 1.

ESMO/European Sarcoma Network Working Group. (2014). Bone sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 25(suppl 3), iii113-iii123.

Campanacci, M. (2013). Bone and soft tissue tumors: clinical features, imaging, pathology and treatment. Springer Science & Business Media.

Burningham, Z., Hashibe, M., Spector, L., & Schiffman, J. D. (2012). The epidemiology of sarcoma. Clinical Sarcoma Research, 2(1), 1.

Ganjoo, K. N., & Patel, S. (2013). The treatment outcome for adult patients with Ewing’s sarcoma. Current Oncology Reports, 15(4), 372-377.

Lin, P. P., Herzog, C. E., Guadagnolo, A., & Patel, S. (2013). Ewing Sarcoma. In Bone Sarcoma (pp. 99-116). Springer US.

Luetke, A., Meyers, P. A., Lewis, I., & Juergens, H. (2014). Osteosarcoma treatment–where do we stand? A state of the art review. Cancer treatment reviews, 40(4), 523-532.

Mason, G. E., Aung, L., Gall, S., Meyers, P. A., Butler, R., Krüg, S., ... & Gorlick, R. (2013). Quality of life following amputation or limb preservation in patients with lower extremity bone sarcoma. Front Oncol, 3(210.10), 3389

Maheshwari, A. V., & Cheng, E. Y. (2010). Ewing sarcoma family of tumors. Journal of the American Academy of Orthopaedic Surgeons, 18(2), 94-107.

Samartzis, D., Nishi, N., Hayashi, M., Cologne, J., Cullings, H. M., Kodama, K., ... & Kasagi, F. (2011). Exposure to ionizing radiation and development of bone sarcoma: new insights based on atomic-bomb survivors of Hiroshima and Nagasaki. J Bone Joint Surg Am, 93(11), 1008-1015.

Sangiolo, D., Mesiano, G., Gammaitoni, L., Leuci, V., Todorovic, M., Giraudo, L., ... & Sarotto, I. (2014). Cytokine-induced killer cells eradicate bone and soft-tissue sarcomas. Cancer Research, 74(1), 119-129.

Stacchiotti, S., Tamborini, E., Vullo, S. L., Bozzi, F., Messina, A., Morosi, C., ... & Palassini, E. (2013). Phase II study on lapatinib in advanced EGFR-positive chordoma. Annals of Oncology, mdt117.

van Oosterwijk, J. G., Anninga, J. K., Gelderblom, H., Cleton-Jansen, A. M., & Bovée, J. V. (2013). Update on targets and novel treatment options for high-grade osteosarcoma and chondrosarcoma. Hematology/oncology clinics of North America, 27(5), 1021-1048.

Wagner, M. J., Livingston, J. A., Patel, S. R., & Benjamin, R. S. (2016). Chemotherapy for Bone Sarcoma in Adults. Journal of Oncology Practice, 12(3), 208-216.

Zhu, L., McManus, M. M., & Hughes, D. P. (2013). Understanding the biology of bone sarcoma from early initiating events through late events in metastasis and disease progression. Front Oncol, 3(230), 1-17.

Zuppinger, A. (2012). Radiation therapy of sarcoma of the bone and soft tissue. American Journal of Roentgenology.

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