It is a rare type of cancer that starts in the bones

By Jennifer Welsh 

Published on June 19, 2023

 Medically reviewed by Archana Sharma, DO

Bone cancers are tumors of abnormal bone and cartilage cells that can spread to other tissues and organs. It can develop from any of the cells in the bone. Many types of bone cancer exist, including osteosarcoma, Ewing's sarcoma, chondrosarcoma, and chordoma.

These malignant primary bone tumors, or bone cancers, are less common than bone tumors that are benign (nonmalignant, noncancerous). They're also less common than cancers that have spread to the bone from other body parts.¹

Bone cancers are rare. The National Cancer Institute predicted 3,970 new cases of bone cancers in 2023, making up about 0.2% of all cancer cases.² About 2,160 of these cases will be in males and 1,810 in females.³ The terms for sex or gender from the source are used when citing research or health authorities.

Bone cancer is more common in children than adults. It accounts for about 5% of all childhood cancers.⁴

This article will cover the signs and symptoms of bone cancer, how long a person can have bone cancer without having symptoms, and the survival rate of several types of bone cancer.

Luis Alvarez / Getty Images

Symptoms of Bone Cancer

There are few initial warning signs of bone cancer. People often do not know they have bone cancer. 

Pain in the bone is the most common sign of bone cancer.⁵ Not all bone cancers cause pain. The pain can come and go, worsen at night or when the bone is used, and isn’t relieved by over-the-counter pain medicine.⁵

Other symptoms of bone cancer are:⁴¹

• Swelling 

• A soft and warm lump in the arms, legs, chest, or pelvis 

• A lump in the back of the throat that causes trouble swallowing or breathing⁵

• Unexplained fever

• Broken bones without a clear cause, leading to sudden, intense pain⁵

• Fatigue

• Weight loss

• Anemia (not enough healthy red blood cells)

• Numbness, tingling, or weakness in different parts of the body if the tumor is pushing on a nerve⁵

Many of these symptoms are more likely caused by other conditions. Talk to a healthcare provider if you have these symptoms.⁴

What Are the Main Types of Bone Cancer?

There are several main types of bone cancer and a few not-too-common ones. All of these cancers are rare. They fall into a category of cancers called bone sarcomas.¹

Sarcomas are cancers that can develop in almost any place in the body. They include bone, muscle, connective tissue, blood vessels, and fat tissue cancers.⁶

Bones are not inert. Rather, they are complex tissues that are constantly changing. They contain many different types of cells and tissues. Bone starts as a flexible tissue called cartilage. Bone forms as the body lays down calcium onto cartilage. Cartilage can remain at the ends of the bones, cushioning the joints. Cartilage also comprises the nose, ears, windpipe, and voice box.

Two sets of cells continuously turn over bone. Cells that lay down new bone are called osteoblasts, and cells that dissolve old bone are called osteoclasts.

Bone marrow comprises fatty tissue and tissues that make new red blood cells, white blood cells, and blood platelets. Other bone marrow cells include fibroblasts, which build connective tissue. Any of these bone cells can develop into bone cancer.

Secondary Bone Cancer

Cancer that spreads to the bones from other body parts is called secondary or metastatic bone cancer. It retains the name of the cancer where it began (such as metastatic breast cancer). Secondary bone cancer is much more common than primary bone cancer.¹

Osteosarcoma, for example, develops from osteoblasts. Chondrosarcoma comes from cartilage cells. Ewing's sarcoma comes from nerve cells in the bone marrow.⁴

Generally, osteosarcoma is the most common primary bone cancer, then chondrosarcoma and Ewing's sarcoma. But this depends on how old a person is. Chondrosarcoma is the most common primary bone cancer in adults, followed by osteosarcomas, chordomas, and Ewing's sarcoma tumors. Osteosarcoma and Ewing's sarcoma are much more common in kids and teens.³

Read on for more details about these different types of bone cancers.

Osteosarcoma 

Osteosarcoma (osteogenic sarcoma) is a cancer that develops in the osteoblasts of immature bone tissue. It’s the most common type of bone cancer.⁶

In young people, it typically appears in the arm bone near the shoulder or the leg bone near the knee. It’s most common in children, teens, and young people between ages 10 and 30. Males are more likely to develop it than females. In young people, it's more common in Black children and children of some other racial groups and is not as common in White children.¹

 Osteosarcoma

About 10% of osteosarcomas develop in adults over age 60,⁶ which can appear in any bone. Whites are more likely to get osteosarcoma as adults than other racial groups.¹

 Signs and Symptoms of Osteosarcoma

Ewing's Sarcoma

Ewing's sarcoma is the second most common type of bone cancer in children and teens under 19. It’s more common in boys than girls. It is much more common in Whites than in other racial groups. These tumors are rare in adults older than 30 but are still the third most common type of bone cancer.¹

It develops in bone and sometimes in other tissues like muscle, fat, connective tissue, or blood vessels. In the bones, it most often appears in the hips, legs, ribs, shoulder blades, and spine but can form in any bone. It can grow and spread quickly to other body parts, often to the lungs.⁶

 The Symptoms of Ewing's Sarcoma

Chondrosarcoma

Chondrosarcoma is a bone cancer that starts in cartilage, most often the cartilage of the pelvis, upper leg, and shoulder.¹ It can also begin in the arms, trachea, larynx, chest wall, ribs, or skull.⁶

These tumors develop primarily in adults 40 and over, becoming more common with advanced age.¹ It’s the second most common primary bone cancer overall.⁶ It’s typically a slow-growing tumor. However, sometimes it can be aggressive and spread to body parts.¹

 Symptoms of Chondrosarcoma

Chordoma

A chordoma is a very rare type of bone cancer. It develops in the spinal bones, usually at the spine's base or the skull's bottom. 

It is most common in adults age 30 and over⁶ and seems to develop about twice as often in men than in women.¹ Rarely does it show up in children and teens.

These tumors often grow slowly and seldom spread to other body parts. But they can come back if not removed completely.⁶

Less Common Types of Bone Cancer

Several other types of cancer can develop in the bone tissue. These include:

• Fibrosarcoma starts in the connective tissue–building cells. It most commonly grows in soft tissues in middle-aged adults. It can grow in the legs, arms, and jaw bones.⁶

• Giant cell tumor of bone is a cancer of the bone-eating cells (osteoclasts) and is also called osteoclastoma. Most often benign, giant cell tumors form at the ends of the long bones of the arms and legs. They develop in people in their 20s and 30s.⁶ They can destroy nearby bone tissue and come back after surgery. Rarely do they spread to other body parts.¹

• Osteoblastoma is a cancer of the bone-building cells. This tumor replaces bone tissue with a weaker form called osteoid. Osteoblastoma most often develops in the spine in young and middle-aged adults. It is slow-growing, rarely spreading to other organs.¹

• High-grade undifferentiated pleomorphic sarcoma of bone is a type that usually grows in the connective tissues and is rare in bones. In the bone, it typically affects the leg (often around the knees) or arm bones. It’s most common in older and middle-aged adults. It often spreads nearby. Sometimes, it spreads to distant body parts. It was previously known as malignant fibrous histiocytoma of bone.⁶

Causes and Risk Factors

There is no common cause of bone cancer. Risk factors include treatments like radiation, chemotherapy, or a stem cell transplant.

Risks are also higher in inherited conditions like hereditary retinoblastoma,  Li-Fraumeni syndrome, hereditary defects of bones, and tuberous sclerosis complex. Specific benign bone conditions like Paget's disease of bone can also increase your risk for bone cancer.¹

How Is Bone Cancer Diagnosed?

Bone cancer is diagnosed by a healthcare provider taking your health history and asking about cancer in your family and then ordering blood and imaging tests. These tests will likely include:¹

• X-rays and other special imaging tests, like bone scans, computed tomography (CT) scans, magnetic resonance imaging (MRI), positron-emission tomography (PET) scans, and angiograms

• Biopsy to take a sample of the tumor, which uses a needle or surgery to remove the cancer or takes a sample that is then analyzed in the lab

• Blood tests for alkaline phosphatase and lactate dehydrogenase (LDH), compounds that indicate that bone tissue is growing⁴

 Causes and Risk Factors of Osteosarcoma

Treatment Options for Bone Cancer

If you're diagnosed with bone cancer, seek care at a specialty sarcoma center with experience treating these rare cancers.

In many cases, bone cancer can be cured, especially if caught early and in a location that makes the tumor easy to remove entirely with surgery.  Even bone cancer that has spread to the lungs can be cured in some cases if the metastases are removed.⁷

The specific treatment plan for your bone cancer depends on the type of cancer it is, the location of the tumor, the cancer's stage and grade, as well as your age and general health.⁴ Ask your care team for more information on what treatments would be the most effective for your case.

Bone cancer is treated with surgery, chemotherapy, radiation, and targeted therapy.¹ New treatments for these rare cancers can be found through clinical trials. 

Surgery

If the cancer is caught early and seems confined to where it started, surgery is the primary treatment. The goal of surgery is to remove the tumor entirely, making sure all of the cancer cells are gone.

Sometimes this involves removing a limb, but more than 90% of people with bone cancer in their limbs can be treated with limb-sparing surgery and keep their limbs. They may need further reconstructive surgery to improve the look and function of the limb.⁷

If the cancer is more advanced or looks aggressive, you'll likely need surgery and other treatments, like chemotherapy or radiation.

 Chemotherapy Side Effects

Chemotherapy

Chemotherapy drugs kill fast-growing cells, like cancer cells. Many different drugs are approved to treat bone cancer or are being studied in clinical trials. A few of these are:

• Cosmegen (dactinomycin) for Ewing's sarcoma⁸

• Doxorubicin hydrochloride for metastatic bone cancer⁹

• Ifex (ifosfamide) for Ewing's sarcoma and osteosarcoma¹⁰

• Trexall (methotrexate sodium) for osteosarcoma¹¹

Targeted Therapy

Targeted therapies fight cancers based on their characteristics. Some bone cancers may have changes in the NTRK gene that can be targeted by the drugs Vitrakvi (larotrectinib) or Rozlytrek (entrectinib).⁷

Bone-Strengthening Drugs

Bone-strengthening drugs might also help treat bone cancers. These drugs have been approved to treat bone cancer:¹¹

• Prolia (denosumab)

• Reclast (zoledronic acid)

Radiation Therapy

Radiation therapy can shrink the tumor in cases of bone cancer that can’t be removed with surgery. It may also be used after surgery to destroy any remaining cancer cells. Shrinking a tumor with radiation before surgery may help surgeons spare a limb with less-extensive surgery.⁷

Outlook for Bone Cancer

The survival rate for bone cancer depends on the type of bone cancer, where it is in the body, if it has spread, how well it responds to treatment, and the person’s overall health and age.³

According to the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program database, the five-year relative survival rate for bone and joint cancers between 2013 and 2019 was 68.9%.² The five-year survival rate is much higher for bone cancer diagnosed while it is still localized.

About 2,140 people will die of bone cancer in 2023, about 0.4% of all cancer deaths.² About 1,200 deaths will be of males and 940 of females.³

Osteosarcoma

The five-year relative survival rate for people with osteosarcoma between 2012 and 2018 were:¹²

• 76% if the cancer is still confined to the bone

• 64% for osteosarcoma that has spread to local tissues or lymph nodes

• 24% when cancer has spread to other organs 

• 59% overall

Factors linked to a better prognosis include younger age, being female, having a primary tumor that develops in the limbs (arms or legs), cancer that can be completely removed with surgery, normal blood levels of alkaline phosphatase and LDH levels, and a good response to chemotherapy.¹²

Ewing's Sarcoma

The five-year relative survival rates for Ewing's sarcoma between 2012 and 2018 were:¹³

• 82% for Ewing's sarcoma just in the bone

• 71% when it has spread to local tissues or lymph nodes

• 30% for Ewing's sarcoma that has spread to other organs

• 63% overall

For Ewing's sarcoma, the factors linked with a better prognosis include smaller tumor size, younger age (under 10), the tumor is in the arm or leg, normal levels of LDH, and a good response to chemotherapy.¹³

Chondrosarcoma

The five-year relative survival rates for people with chondrosarcoma between 2012 and 2018 were:¹⁴

• 91% when chondrosarcoma was still confined to the cartilage

• 76% if it spread to local tissues or lymph nodes

• 17% when chondrosarcoma has spread to other organs

• 79% overall

Chordoma

The five-year relative survival rate for people with chordoma between 2012 and 2018 was:¹⁴

• 87% when chordoma is still confined to the spine

• 84% when chordoma has spread to local tissues or lymph nodes

• 69% for chordoma that has spread to other organs

• 84% overall

Giant Cell Tumor of Bone

The five-year relative survival rate of people with giant cell tumors of bone in 2012 and 2018 was:¹⁴

• 90% if the cancer is still confined to the bone

• 77% if the tumor has spread to local tissues or lymph nodes

• 36% if the cancer spread to other organs

• 78% overall

Summary

Bone cancer is tumors that develop from abnormal bone and cartilage cells. They are cancerous because they can spread to other tissues and organs. They fall into a category of cancer called sarcomas.

Bone cancer is rare, accounting for about 0.2% of all cancer cases. Bone cancers are less common than benign bone tumors. They are also less common than secondary bone cancers that have spread to the bone from other body parts.

Common types of bone cancer include osteosarcoma, Ewing's sarcoma, chondrosarcoma, and chordoma. Osteosarcoma is the most common type. It typically affects young people. Ewing's sarcoma is the second most common type in children and teens. Chondrosarcoma is more common in adults.

Bone cancer symptoms include pain, swelling, a lump, or broken bones without a clear cause. Someone with bone cancer may also have fevers, fatigue, weight loss, or anemia. Bone cancer may cause numbness or weakness if a tumor is pressing on a nerve.

Bone cancer is diagnosed using your medical history, an exam, blood and imaging tests, and a biopsy. Bone cancer treatment is typically surgery. It may also include chemotherapy, radiation, and targeted therapy.

People with bone cancer should get specialized care at a sarcoma center. They may also offer clinical trials of new treatment options.

The outlook for bone cancer varies depending on various factors, including the type and stage of the cancer. The five-year relative survival rate for bone and joint cancers between 2013 and 2019 was 68.9%.