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Primary Cancerous Bone Tumors

By Michael J. Joyce, MD, Associate Clinical Professor, Orthopaedic Surgery, Cleveland Clinic Lerner School of Medicine at Case Western Reserve University ; Hakan Ilaslan, MD, Associate Professor of Radiology;Staff Radiologist, Cleveland Clinic Lerner College of Medicine at Case Western Reserve University;Imaging Institute, Diagnostic Radiology

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

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.

To diagnose chondrosarcomas, doctors take x-rays and remove a tissue sample for examination under a microscope (biopsy). A bone scan may also be done.

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). Symptoms may exist for months to several years before diagnosis. Chordomas do not usually spread (metastasize) to other bones, but they may return after treatment.

To diagnose chordomas, doctors do magnetic resonance imaging (MRI).

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 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.

To diagnose Ewing sarcoma, doctors take x-rays. Although x-rays can show some details, magnetic resonance imaging (MRI) can help determine the exact size of the tumor. To confirm the diagnosis, doctors remove a tissue sample for examination under a microscope (biopsy).

Treatment of Ewing sarcoma includes various combinations of surgery, chemotherapy, and radiation therapy, depending on whether surgery is practical or, if attempted, successful. These treatment combinations can cure more than 60% of people who have Ewing sarcoma.

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 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).

To diagnose lymphoma of bone, doctors take x-rays.

Treatment of lymphoma of bone usually consists of a combination of chemotherapy with or without radiation therapy, which seems to be as effective as surgical removal of the tumor. Amputation is rarely necessary. If a bone seems as though it may fracture, doctors may stabilize it surgically in an attempt to prevent a fracture.

Malignant giant cell tumors

Malignant giant cell tumors are rare and cancerous and are usually located at the extreme end of a long bone (arm or thigh bone). These typically cause pain and swelling.

To diagnose malignant giant cell tumors, doctors take x-rays.

Treatment of malignant giant cell tumors is similar to that of osteosarcomas, but the cure rate is low.

Multiple myeloma

Multiple myeloma (see also 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.

To diagnose multiple myeloma, doctors take x-rays. Blood tests are also done. To confirm the diagnosis of multiple myeloma, doctors remove and examine bone marrow cells.

Treatment of multiple myeloma is complex and may include chemotherapy, radiation therapy, and sometimes surgery.

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, 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 are taken, but removal of a tissue sample for examination under a microscope (biopsy) is needed for the diagnosis of osteosarcoma. People need a chest x-ray and a chest computed tomography (CT) scan to detect cancer that has metastasized to the lungs and a bone scan to detect cancer that has spread to other bones.

More than 65% of people who have this type of tumor survive for at least 5 years after diagnosis when chemotherapy 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. 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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