Bone Sarcoma

The Penn Medicine Sarcoma Program’s expert team treats all types of bone tumors across a range of ages, including both malignant (cancerous) and benign (noncancerous) tumors that begin in bones.

What Is a Bone Sarcoma?

A bone sarcoma is a primary bone cancer—meaning the cancer starts within the bone tissue and forms a tumor. These tumors can develop in many areas of the body, most often in the arms, legs, and pelvis. Some bone sarcomas grow slowly, while others act more aggressively.

Many tumors found within bone tissue are benign (noncancerous). Bone sarcomas that are cancerous are actually quite uncommon, accounting for less than 1 percent of all diagnosed cancers.

Types of Bone Sarcoma

Our team provides care for all types of tumors found in the bones. Bone sarcomas include, but are not limited to:

  • Adamantinoma, a tumor most often found in the tibia (lower leg)
  • Angiosarcoma, an aggressive blood vessel tumor that can very rarely be found in the bone
  • Chondrosarcoma, a tumor that usually grows slowly, most often in the bones of the pelvis, humerus (upper arm), and femur (thigh)
  • Chordoma, a slow-growing tumor in the spine, primary at the base of the skull or lower spine (sacrum)
  • Ewing sarcoma, the second most common bone cancer in children and usually found in the bony pelvis as well as the femur and humerus
  • Leiomyosarcoma, a sarcoma that rarely starts in the bone
  • Osteosarcoma, the most common bone cancer in children and most commonly found around the bones of the knee as well as the humerus and pelvis
  • Undifferentiated pleomorphic sarcoma (UPS), a sarcoma that more commonly starts in the soft tissue but rarely can start in the bones

Bone Cancer Symptoms

People with bone sarcomas often feel constant pain that gets worse over time. Bone sarcomas can also break out of the bone into the surrounding soft tissues or lead to fractures. In the spine, they can press on nerves and cause pain.

Causes of Bone Sarcomas

The causes of most bone sarcomas are currently unknown, however researchers are continuing to study them.

In rare cases, radiation therapy and chemotherapy for other cancers can lead to the later development of bone cancer. Some people with noncancerous bone conditions called Paget disease may develop osteosarcoma.

Some genetic variants (changes), which are inherited, can be tied to rare syndromes. These syndromes cause various symptoms and diseases and can raise the risk for some cancers, including bone sarcomas. The genetic syndromes tied to higher bone cancer risk include:

  • Bloom syndrome: Osteosarcoma
  • Hereditary retinoblastoma: Osteosarcoma
  • Li-Fraumeni syndrome: Osteosarcoma
  • Multiple osteochondromas: Chondrosarcoma
  • Rothmund-Thomson syndrome II: Osteosarcoma
  • Tuberous sclerosis: Chordoma
  • Werner syndrome: Osteosarcoma

If one of these syndromes runs in your family, specialists at the Abramson Cancer Center can provide genetic testing and counseling.

Bone Sarcoma Diagnosis

A bone sarcoma diagnosis starts with imaging tests, usually an X-ray. If we require more images, we might order magnetic resonance imaging (MRI) as well. Our doctors may also recommend taking a sample of the suspected tumor during a biopsy, for our pathologists to examine.

Learn more about sarcoma diagnosis and staging.

Bone Sarcoma Treatment at Penn Medicine

Almost all bone sarcomas require surgery to remove them for the best overall result. For certain types of bone cancer, including Ewing sarcoma and osteosarcoma, chemotherapy before and after surgery will be required. Ewing sarcoma is a type of sarcoma in the bone that can be treated with either radiation or surgery in addition to the chemotherapy. Chondrosarcomas and chordomas do not respond to chemotherapy or immunotherapy.

Radiation therapy is used in combination with surgery for the treatment of chordoma in the lower spine or at the bottom of the skull. An advanced, highly accurate form of radiation called proton therapy may help in those cases. Occasionally, high-dose radiation is used alone for the treatment of chordoma in certain situations.

Because bone sarcoma is a cancer, it has the possibility to metastasize (spread). For these tumors, we have the expertise to provide a range of treatment options, including emerging therapies only available through clinical trials.

Learn more about sarcoma treatment.

Make an Appointment

Please call 800-789-7366 or make an appointment.

The Penn Sarcoma Program offers same-day appointments to patients who have their imaging available. To learn more, call 215-349-8200.