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

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.

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 Penn Medicine can provide genetic testing and counseling.

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.