Topic Resources
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 of Cancer.)
Biopsy

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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 diagnose adamantinomas, doctors take x-rays and remove a tissue sample for examination under a microscope (biopsy).
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.
To diagnose chondrosarcomas, doctors take x-rays and do a bone scan and MRI. Doctors also remove a tissue sample for examination under a microscope (biopsy).
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.
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 do a biopsy.
Ewing Sarcoma of Bone

Image courtesy of Michael J. Joyce, MD, and Hakan Ilaslan, MD.
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).
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 and also do MRI and a biopsy.
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.
Bone biopsy is done sometimes for diagnosis in areas where bone
has been destroyed. If multiple myeloma is suggested by the bone biopsy
results or if multiple myeloma is suspected for other reasons, the
diagnosis is confirmed by removing and examining bone marrow cells.
Blood tests are also done. Additionally, doctors take x-rays
of the entire body (skeletal survey). Magnetic resonance imaging (MRI)
or positron emission tomography (PET) combined with computed tomography
(PET-CT) may also be done to examine specific sites of bone pain.
Multiple Myeloma

Image courtesy of Michael J. Joyce, MD, and Hakan Ilaslan, MD.
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. Magnetic resonance imaging (MRI) and positron emission tomography (PET) combined with computed tomography (PET-CT) are other imaging tests that are also done.
Osteosarcoma

Image courtesy of Michael J. Joyce, MD, and Hakan Ilaslan, MD.
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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