All About Sarcomas of the Bone

Author: OncoLink Team
Last Reviewed: July 6, 2018

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. 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.

What causes sarcoma of the bone (SB) and am I at risk?

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 3,450 new cases annually in the bone. SB’s occur most frequently in people under the age of 44, with 27.3% of all cases occurring in children and adolescents under the age of 20.

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, providers do not know why SBs occur and therefore cannot determine who is at risk of developing the disease.

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, osteosarcoma and Ewing sarcoma are the most common types. The rates of osteosarcoma and Ewing sarcoma are the same for boys and girls until 13 years of age, after which males are more commonly affected. 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 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 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 sarcoma family of tumors and are treated similarly.

How can I prevent SB?

As with all cancers, not smoking, maintaining a healthy weight and exercise are important in prevention. There are no specific prevention methods for SB.

What screening tests are used for SB?

Unfortunately, there are no screening tests for SB. 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 SB?

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 is SB diagnosed?

Given how rare sarcomas are, many providers have never seen or cared for a patient with sarcoma. When sarcoma is suspected, it is important to seek out a care 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. A CT scan of the chest is usually done to rule out any metastases (spread) to the lungs (this is the most common site of tumor spread). MRI (magnetic resonance imaging) with or without a CT scan will be done in preparation for surgery. In some cases, a 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 is SB staged?

The most widely used system has been developed by the American Joint Committee on Cancer (AJCC) (8th 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. 

The staging system is very complex, and the entire staging system is outlined at the end of this article. Though complicated, the staging system helps healthcare providers determine the extent of the cancer, and in turn, make treatment decisions for a patient's cancer.  

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. This staging system is also located at the end of this article. 

How is SB treated?

Given the rarity of SB, these patients are best served at a specialty treatment center. 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 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. 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. In Ewing sarcoma, ifosfamidecyclophosphamideetoposidedoxorubicin and vincristine are the most effective medications. In osteosarcoma, doxorubicincisplatincarboplatinifosfamide, epirubicin and methotrexate are more effective. Osteosarcomas that are relapsed or metastatic may also be treated with gemcitabine, sorafenib or everolimus. These medications are generally used in combinations of several drugs that work in different ways.

Mesenchymal chondrosarcoma is treated using Ewing sarcoma regimens. Dedifferentiated chondrosarcoma is treated following osteosarcoma regimens.

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 the medications used in the treatment of chordomas are lapatinib(for EGFR positive chordomas), erlotinibimatinibsunitinib and sorafenib.

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. Talk to your provider about participating in clinical trials in your area. You can also explore currently open clinical trials using the OncoLink Clinical Trials Matching Service.

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. Recommendations for follow up care are based on the type of sarcoma:

Osteosarcoma

Follow-up care should include physical exam by both orthopedics and oncology, 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. Your provider may also order laboratory studies and a PET/CT scan or bone scan. 

Chondrosarcoma

Follow-up care should consist of physical exam, chest x-ray, and x-ray of primary tumor site. For low grade tumors follow-up care should occur every 6 months for 2 years, then annually. For high grade tumors your care team may order an MRI or CT of the primary site in addition to other radiologic exams every 6 months for 5 years, then annually for a minimum of 10 years.

Chordoma

Follow-up care should include physical exam, x-ray or CT/MRI of the surgical site, chest x-ray every six months for 5 years and then annually. You should also have a CT of your abdomen and pelvis every year.

Ewing's Sarcoma

Follow-up care should include physical exam, MRI and/or CT of the primary site and chest x-ray every 2-3 months for 2 years. Over time the span between visits can expanded to a goal of annual exams after 5 years.  Your care team may order laboratory tests and a PET/CT scan depending on your case.

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.

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 mission of "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/

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/

Appendix

AJCC Staging System for Soft Tissue Sarcomas (8th Edition 2016)

Primary Tumor (T)

Appendicular Skeleton, Trunk, Skull, and Facial Bones

 

Description

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

 

Primary Tumor (T)

Spine*

Description

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Tumor confined to one vertebral segment or two adjacent vertebral segments

T2

Tumor confined to three adjacent vertebral segments

T3

Tumor confined to four or more adjacent vertebral segments, or any nonadjacent vertebral segments

T4

Extension into the spinal canal or great vessels

T4a

Extension into the spinal canal

T4b

Evidence of gross vascular invasion or tumor thrombus in the great vessels

 

Primary Tumor (T)

Pelvis*+

Description

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Tumor confined to one pelvic segment with no extraosseous extension

T1a

Tumor ≤8 cm in greatest dimension

T1b

Tumor >8 cm in greatest dimension

T2

Tumor confined to one pelvic segment with extraosseous extension or two segments without extraosseous extension

T2a

Tumor ≤8 cm in greatest dimension

 

T2b

Tumor >8 cm in greatest dimension

T3

Tumor spanning two pelvic segments with extraosseous extension

T3a

Tumor ≤8 cm in greatest dimension

T3b

Tumor >8 cm in greatest dimension

T4

Tumor spanning three pelvic segments or crossing the sacroiliac joint

T4a

Tumor involves sacroiliac joint and extends medial to the sacral neuroforamen

T4b

Tumor encasement of external iliac vessels or presence of gross tumor thrombus in major pelvic vessels.

*There are no AJCC prognostic stage groupings for spine and pelvis

Regional Lymph Nodes (N)

Description

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)

Description

M0

No distant metastasis

M1

Distant metastasis

M1a

Lung metastasis

M1b

Bone or other distant sites

 

Histologic Grade (G)

Description

GX

Grade cannot be assessed

G1

Well differentiated-low grade

G2

Moderately differentiated-low grade

G3

Poorly differentiated

 

Stage Grouping

T

N

M

G

Stage IA

T1

N0

M0

G1, GX

Stage IB

T2

N0

M0

G1, GX

T3

N0

M0

G1, GX

Stage IIA

T1

N0

M0

G2, G3

Stage IIB

T2

M0

M0

G2, G3

StageIII

T3

N0

M0

G2, G3

Stage IVA

Any T

N0

M1a

Any G

Stage 4B

Any T

N1

Any M

Any G

Any T

Any N

M1b

Any G

 

Surgical Staging System (SSS)-Stage

Grade

Site

IA

Low (G1)

Intracompartmental (T1)

IB

Low (G1) 

Extracompartment (T2)

IIA

High (G2)

Intracompartmental (T1)

IIB

High (G2)

Extracompartmental (T2)

III

Any G + regional or distant metastasis

Any (T)

 

Types of Sarcoma of the Bone

(This list does not contain all types)

  • Adamantinoma
  • Angiosarcoma
  • Chondrosarcoma
  • Chordoma
  • Clear cell chondrosarcoma
  • Classic steosarcoma
  • Ewing 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
  • Periosteal chondroma
  • Periosteal osteosarcoma
  • Peripheral neuroectodermal tumor (PNET)
  • Primitive neuroectodermal tumor of bone
  • Small cell osteosarcoma
  • Telangiectatic osteosarcoma

References

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

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

The American Cancer Society. Facts and Figureswww.cancer.org

Abeloff, M., Armitage, J., Niederhuber, J., Kastan, M. & McKenna, G. (Eds.): Clinical Oncology (2004). Elsevier, Philadelphia, PA.

Carvajal, R. & Meyers, P. (2005) "Ewing 's sarcoma and primitive neuroectodermal family of tumors" Hematol Oncol Clin North Am 19 (3): 501-525.

Burningham, Z., Hashibe, M., Spector, L., & Schiffman, J. D. (2012). The epidemiology of sarcoma. Clinical Sarcoma Research2(1), 1.

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

ESMO/European Sarcoma Network Working Group. (2014). Bone sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology25(suppl 3), iii113-iii123.

Ganjoo, K. N., & Patel, S. (2013). The treatment outcome for adult patients with Ewing’s sarcoma. Current Oncology Reports15(4), 372-377.

Gustafson, P., K. E. Dreinhofer, et al. (1994). "Soft tissue sarcoma should be treated at a tumor center. A comparison of quality of surgery in 375 patients." Acta Orthop Scand 65 (1): 47-50..

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 reviews40(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 Oncol3(210.10), 3389

Maheshwari, A. V., & Cheng, E. Y. (2010). Ewing sarcoma family of tumors. Journal of the American Academy of Orthopaedic Surgeons18(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 Am93(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 Research74(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.

Steffner, R. J., & Jang, E. S. (2018). Staging of Bone and Soft-tissue Sarcomas. JAAOS-Journal of the American Academy of Orthopaedic Surgeons26(13), e269-e278.

Strauss, S. J., & Whelan, J. S. (2018). Current questions in bone sarcomas. Current opinion in oncology30(4), 252-259.

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 America27(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 Practice12(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 Oncol3(230), 1-17.

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

Keywords

Click on any of these terms for more related articles

Blogs

Today is Rare Disease Day!
by Christina Bach, MSW, LCSW, OSW-C
February 28, 2019

Thank You, Fati.
by Christina Bach, MSW, LCSW, OSW-C
January 31, 2019

A
B
C
D
E
F
G
H
I
J
K
L
M
N
O
P
R
S
T
U
V
X
Y
Z
#
 
A
B
C
E
F
G
H
K
L
M
N
O
P
R
S
T
U
V
 
 
Stay informed with the latest information from OncoLink!   Subscribe to OncoLink eNews
View our newsletter archives