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Primary Cancerous Bone Tumors

By

Michael J. Joyce

, MD, Cleveland Clinic Lerner School of Medicine at Case Western Reserve University;


Hakan Ilaslan

, MD, Cleveland Clinic Lerner College of Medicine at Case Western Reserve University

Last full review/revision Jun 2020| Content last modified Jun 2020
Click here for the Professional Version
Topic Resources

Tumors that originally start in the bone are called primary bone tumors. Primary bone tumors may be noncancerous (benign) or cancerous (malignant).

Adamantinomas

Adamantinomas are rare tumors that most often develop in the shinbone (tibia). The tumors usually occur in adolescents and people who are in their 20s but can occur at any age. They often cause pain, and people often can feel the tumor beneath the skin when they run their fingers over it.

These tumors grow slowly and are low-grade cancers, which means they are less likely to spread (metastasize) than some other tumors. However, although rare, metastases do occur (mostly to the lungs).

To treat adamantinomas, doctors surgically remove them without cutting into the tumor, which risks spilling the tumor cells. If the cells spill, the cancer can return. On rare occasions, surgical removal of the affected leg (amputation) may be necessary depending on the location of the tumor or depending on whether the tumor returns.

Chondrosarcomas

Chondrosarcomas are tumors composed of cancerous cartilage cells. These tumors tend to occur in older adults. These tumors often develop in bones such as the pelvis or shoulder blade (scapula) but can develop in any portion of any bone and can also develop in the tissues surrounding the bones. Many chondrosarcomas are slow-growing or low-grade tumors, meaning that they are less likely to spread (metastasize) than some other tumors. However, some chondrosarcomas are fast-growing or high-grade tumors, which tend to metastasize.

Low-grade chondrosarcomas are often removed from the bone by scraping it with a scoop-shaped instrument (curettage) and by using liquid nitrogen, phenol, bone cement (methyl methacrylate), or an argon beam to kill the surface tumor cells embedded in the bone. Nearly all low-grade tumors are cured with these surgical treatments.

High-grade or fast-growing chondrosarcomas are aggressive tumors and are more likely to metastasize than some other tumors. They must be completely removed surgically without cutting into the tumor, which risks spilling the tumor cells. If the cells spill, the cancer can return.

Chondrosarcomas of any grade do not respond to chemotherapy or radiation therapy. Surgical removal of the affected arm or leg (amputation) is rarely necessary.

Chordomas

Chordomas are rare and cancerous and tend to occur at the ends of the spinal column, usually in the middle of the base of the spine (sacrum) or tailbone or near the base of the skull. A chordoma affecting the sacrum or tailbone causes nearly constant pain. A chordoma in the base of the skull can cause problems in nerves at the base of the skull (the cranial nerves Overview of the Cranial Nerves Twelve pairs of nerves—the cranial nerves—lead directly from the brain to various parts of the head, neck, and trunk. Some of the cranial nerves are involved in the special senses (such as seeing... read more ). Symptoms may exist for months to several years before diagnosis. Chordomas do not usually spread (metastasize) to other areas such as the lung unless they are more aggressive, but they may return after treatment.

Chordomas affecting the sacrum or tailbone may be cured by surgical removal. Chordomas in the base of the skull usually cannot be cured surgically, but radiation therapy Radiation Therapy for Cancer Radiation is a form of intense energy generated by a radioactive substance, such as cobalt, or by specialized equipment, such as an atomic particle (linear) accelerator. Radiation preferentially... read more may temporarily shrink the tumor and help with pain.

Ewing sarcoma of bone

Ewing sarcoma is a cancerous tumor that affects males more often than females and appears most commonly in people aged 10 to 25 years. Most of these tumors develop in the arms or legs, but they may develop in any bone. Pain and swelling are the most common symptoms. Tumors may become quite large, sometimes affecting the entire length of a bone. The tumor may include a large mass of soft tissue.

Fibrosarcomas and undifferentiated pleomorphic sarcomas of bone

Fibrosarcomas and undifferentiated pleomorphic sarcomas of bone (formerly known as malignant fibrous histiocytoma of bone) affect the same age group as and are similar to osteosarcomas Osteosarcomas (osteogenic sarcoma) Tumors that originally start in the bone are called primary bone tumors. Primary bone tumors may be noncancerous (benign) or cancerous (malignant). (See also Overview of Bone Tumors and Overview... read more Osteosarcomas (osteogenic sarcoma) in appearance, location, and symptoms. These cancerous tumors have cells that produce cancerous fibrous tissue (connective tissue) rather than cancerous bony tissue.

Treatment and survival rates are similar to that of osteosarcoma.

Lymphoma of bone

Lymphoma of bone (previously called reticulum cell sarcoma) is a cancerous tumor that usually affects people in their 40s and 50s. It can originate in any bone or elsewhere in the body and then spread diffusely to bone marrow. Usually, this tumor causes pain and swelling and an accumulation of soft tissue. The damaged bone tends to break (fracture).

Malignant giant cell tumors

Multiple myeloma

Multiple myeloma (see also Plasma Cell Disorders: Multiple Myeloma Multiple Myeloma Multiple myeloma is a cancer of plasma cells in which abnormal plasma cells multiply uncontrollably in the bone marrow and occasionally in other parts of the body. People often have bone pain... read more Multiple Myeloma ) is the most common primary cancerous (malignant) bone tumor and occurs mostly in older adults. However, it is cancer that involves the bone marrow (the blood-forming tissue inside the cavity of the bone) rather than the hard tissue that makes up the bone. Thus, it is usually considered a cancer of the bone marrow rather than the bone itself. It is more common than cancers of the hard tissue that makes up bone.

The cancerous marrow cells secrete substances that cause loss of bone. Bone loss may be widespread or, more often, appears as punched-out areas in bones.

Multiple myeloma may affect one or more bones, so pain may occur in one location or in several. If only one bone is affected by a single tumor, the condition is called plasmacytoma. If there is more than one tumor or the bone marrow is widely affected, the condition is called multiple myeloma.

Osteosarcomas (osteogenic sarcoma)

Osteosarcoma is the second most common type of primary cancerous bone tumor. Although most common among people aged 10 to 25 years, osteosarcomas can occur at any age. Older people who have Paget disease of bone Paget Disease of Bone Paget disease of bone is a chronic disorder of the skeleton in which areas of bone undergo abnormal turnover, resulting in areas of enlarged and softened bone. The breakdown and formation of... read more Paget Disease of Bone , have undergone bone radiation, or have areas of dead bone tissue (called bone infarcts) and other conditions sometimes develop this type of tumor. Osteosarcomas usually develop in or around the knee, but they can originate in any bone. They tend to spread (metastasize) to the lungs or other bones. Usually, these tumors cause pain and swelling.

X-rays X-rays A doctor can often diagnose a musculoskeletal disorder based on the history and the results of a physical examination. Laboratory tests, imaging tests, or other diagnostic procedures are sometimes... read more X-rays are taken, but removal of a tissue sample for examination under a microscope (biopsy Diagnosis Bone tumors are growths of abnormal cells in bones. Bone tumors may be cancerous (malignant) or noncancerous (benign). Cancerous tumors may start in the bone (primary cancer) or start in other... read more ) is needed for the diagnosis of osteosarcoma. People need a chest x-ray Chest Imaging Chest imaging studies include X-rays Computed tomography (CT) CT angiography Magnetic resonance imaging (MRI) read more and a chest computed tomography Computed Tomography (CT) In computed tomography (CT), which used to be called computed axial tomography (CAT), an x-ray source and x-ray detector rotate around a person. In modern scanners, the x-ray detector usually... read more Computed Tomography (CT) (CT) scan to detect cancer that has metastasized to the lungs and a bone scan Bone scanning A doctor can often diagnose a musculoskeletal disorder based on the history and the results of a physical examination. Laboratory tests, imaging tests, or other diagnostic procedures are sometimes... read more Bone scanning to detect cancer that has spread to other bones. Magnetic resonance imaging Computed tomography (CT) and magnetic resonance imaging (MRI) A doctor can often diagnose a musculoskeletal disorder based on the history and the results of a physical examination. Laboratory tests, imaging tests, or other diagnostic procedures are sometimes... read more Computed tomography (CT) and magnetic resonance imaging (MRI) (MRI) and positron emission tomography (PET) combined with computed tomography (PET-CT PET computed tomography (PET-CT) Positron emission tomography (PET) is a type of radionuclide scanning. A radionuclide is a radioactive form of an element, which means it is an unstable atom that becomes more stable by releasing... read more PET computed tomography (PET-CT) ) are other imaging tests that are also done.

More than 65% of people who have this type of tumor survive for at least 5 years after diagnosis when chemotherapy Chemotherapy Chemotherapy involves the use of drugs to destroy cancer cells. Although an ideal drug would destroy cancer cells without harming normal cells, most drugs are not that selective. Instead, drugs... read more is given and the cancer has not metastasized. If chemotherapy destroys almost all of the cancer, the chance of surviving at least 5 years is greater than 90%. Because surgical procedures have improved, the affected arm or leg can usually be saved and reconstructed. In the past, the affected limb often had to be amputated.

Osteosarcomas are usually treated with a combination of chemotherapy and surgery Surgery for Cancer Surgery is a traditional form of cancer treatment. It is the most effective in eliminating most types of cancer before it has spread to lymph nodes or distant sites (metastasized). Surgery may... read more . Usually, chemotherapy is given first. Pain often subsides during this phase of treatment. Then the tumor is surgically removed without cutting into the tumor. Cutting into the tumor spills its cells, which can cause the cancer to return in the same area.

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