| ICD-9-CM: |
| 170.0 - | Malignant Neoplasm of Bone and Articular Cartilage; Bone: Ethmoid, Frontal, Malar, Nasal, Occipital, Orbital, Parietal, Sphenoid, Temporal, Zygomatic, Maxilla (Superior), Turbinate, Upper Jaw Bone, Vomer |
| 170.1 - | Malignant Neoplasm of Bone and Articular Cartilage; Mandible; Inferior Maxilla; Jaw Bone NOS; Lower Jaw Bone |
| 170.2 - | Malignant Neoplasm of Bone and Articular Cartilage; Mandible; Vertebral Column, Excluding Sacrum and Coccyx; Spinal Column; Spine: Vertebra |
| 170.3 - | Malignant Neoplasm of Bone and Articular Cartilage; Ribs, Sternum, and Clavicle; Costal Cartilage; Costovertebral Joint; Xiphoid Process |
| 170.4 - | Malignant Neoplasm of Bone and Articular Cartilage; Scapula and Long Bones of Upper Limb; Acromion; Bones NOS of Upper Limb; Humerus; Radius; Ulna |
| 170.5 - | Malignant Neoplasm of Bone and Articular Cartilage; Short Bones of Upper Limb; Carpal; Cuneiform, Wrist; Metacarpal; Navicular, of Hand; Phalanges of Hand; Pisiform; Scaphoid (of Hand); Semilunar or Lunate; Trapezium; Trapezoid; Unciform |
| 170.6 - | Malignant Neoplasm of Bone and Articular Cartilage; Pelvic Bones, Sacrum, and Coccyx; Coccygeal Vertebra; Ilium; Ischium; Pubic Bone; Sacral Vertebra |
| 170.7 - | Malignant Neoplasm of Bone and Articular Cartilage; Bones NOS of Lower Limb; Femur; Fibula; Tibia |
| 170.8 - | Malignant Neoplasm of Bone and Articular Cartilage; Short Bones of Lower Limb; Astragalus [Talus]; Calcaneus; Cuboid; Cuneiform, Ankle; Metatarsal; Navicular (of Ankle); Patella; Phalanges of Foot; Tarsal |
| 170.9 - | Malignant Neoplasm of Bone and Articular Cartilage; Bone and Articular Cartilage, Site Unspecified |
| 198.5 - | Secondary Malignant Neoplasm of Other Specified Sites; Bone and Bone Marrow |
| 238.0 - | Neoplasm of Uncertain Behavior of Bone and Articular Cartilage |
| 731 - | Osteitis Deformans and Osteopathies Associated with Other Disorders Classified Elsewhere |
| 731.1 - | Osteitis Deformans in Diseases Classified Elsewhere |
| A bone tumor is an abnormal growth of cells within the bone that may be noncancerous (benign) or cancerous (malignant). A bone tumor may be primary, originating within the bone itself, or secondary, resulting from the spread (metastasis) of cancer elsewhere in the body, such as from the lung, breast, or prostate.
Benign bone tumors occur more frequently than malignant bone tumors. This type of bone tumor does not spread (metastasize) to other tissues or organs, but remains localized within the bone. Benign bone tumors are named according to the cell type of origin: Bone cells may produce osteomas, cartilage cells may produce chondromas, and tumors arising from both bone and cartilage produce osteochondromas, which are the most common type of benign bone tumor. The majority of benign bone tumors require no treatment, and the remainder can usually be cured with surgery.
Malignant bone tumors may be primary or secondary. They rarely occur as a primary bone tumor (less than 1% of all malignant tumors), but more frequently result from metastasis (secondary bone cancer) from cancer originating elsewhere in the body. Primary bone cancer, or sarcoma, can originate in different types of bone or adjacent soft tissues (muscle, cartilage, nerves, fat, and connective tissue) anywhere in the body.
The most common types of sarcoma are osteosarcoma, chondrosarcoma, fibrosarcoma, and Ewing's sarcoma. Osteosarcomas occur with the highest frequency. They develop primarily at the ends of the bones at the growth plates of children and young adults, and affect the knees, upper arms, and pelvis. Chondrosarcomas arise from cartilage and develop in the legs, arms, pelvis, and ribs; they may also develop from benign osteochondromas. Fibrosarcomas arise from connective tissue (muscle, ligament, or tendon) but may affect the bones of the jaw, arms, and legs. Ewing's sarcoma, the second most common type of bone cancer in children, develops most frequently in the middle (shaft) of long bones, and affects hips, long bones in the thigh and upper arm, and pelvis. There are several other types of sarcoma such as malignant giant cell tumors and chordoma; these rare tumors occur most often in adults over the age of 30.
Osteosarcomas, the most common type of malignant bone cancer, are classified according to their appearance. A low-grade tumor has few dividing cells and a mature cell matrix as viewed under the microscope; it is unlikely to spread to other parts of the body and carries an excellent prognosis. A high-grade tumor has multiple dividing cells and an immature cell matrix; these tumors have a high likelihood of metastasis and usually require both surgery and chemotherapy. Intermediate-grade tumors have a variable prognosis.
Secondary bone cancer occurs when malignant cells from a primary cancer site, such as the kidney, lung, prostate, breast, or thyroid, spread (metastasize) to the bones; although cancer is present in the bones, the cells are those of the organ of origin. Therefore, these secondary cancers are not referred to as bone cancer but instead reference the original organ from which it metastasized, such as metastatic lung cancer. Common sites of secondary bone cancer include the ribs, skull, pelvis, and vertebrae. These bone tumors may not be treated in the same way as primary bone tumors because they occur in the later stages of metastatic cancer of other organ systems.
Two additional types of cancer may also develop within the bones: non-Hodgkin's lymphoma of the bone, and multiple myeloma, which develops in the plasma cells of the bone marrow.
Excessive exposure to radiation, especially at a young age, and combinations of radiation and chemotherapy for other conditions may increase risk of bone cancer. Certain genetic risk factors may be associated with a propensity for the development of sarcomas. An overactive parathyroid gland is also believed to be a risk factor. Individuals treated with certain kinds of anticancer drugs (alkylating agents) may be at increased risk for malignant bone tumors. Individuals who have had multiple benign tumors (osteochondromas) or who have been diagnosed with Paget's disease or who have a long-term bone infection (osteomyelitis) may also be more likely to develop primary sarcomas.Risk: Primary malignant bone tumors are rare, and in most cases the cause is unknown, although research has identified genetic associations. Men are more likely than women to develop osteosarcoma, and more teenagers than adults will be affected (ACS). Osteosarcomas are the most commonly diagnosed in individuals between the ages of 10 and 30 and between the ages of 60 and 80; they are rarely discovered in middle age (ACS). Those with pre-existing bone disease or Paget's disease also have increased risk.
Chondrosarcomas are unusual in those younger than 20 years of age; there is increasing risk from age 20 to age 75, and men and women are equally affected. Fibrosarcomas usually occur in middle-aged and elderly adults.
Ewing's sarcoma occurs primarily in children and teenagers, and is uncommon in adults over the age of 30. Although whites have increased risk for this type of primary bone cancer, it is rare in blacks and Asian-Americans. Incidence and Prevalence: According to the American Cancer Society (ACS) the following apply: An estimated 2,570 cases of malignant bone tumors will be diagnosed in 2005 in the US, with an estimated 1,210 deaths.
The most common type of primary bone cancer is osteosarcoma, accounting for 35% of primary bone cancers. In the US each year, 900 new cases of osteosarcoma are diagnosed.
Chondrosarcoma represents 26% of bone cancer cases. Fibrosarcoma and malignant fibrous histiocytoma account for 6% primary bone cancer and chordoma accounts for 8%. Giant cell tumors make up approximately 10% of malignant bone cancer. And Ewing's sarcoma accounts for 16% of cases of primary bone cancer. |
Source: Medical Disability Advisor
| History: Many benign bone tumors have no symptoms at all, and may be found incidentally.
The most common symptom reported with malignant bone tumors is pain, especially pain that worsens with activity and at night. Other symptoms include swelling, stiffness, tenderness, or a mass that can be felt at the tumor site if the disease has advanced. Bone fractures may have occurred. Individuals with Ewing's sarcoma, a rare malignant tumor of the bone, often report fever and weight loss. Pallor and weight loss may also indicate poor general health stemming from underlying metastatic cancer in another organ. Individuals in later stages of the disease may complain of fever, weight loss, fatigue, difficulty moving, and spontaneous bone fractures as a result of loss of bone integrity. Physical exam: The exam may reveal swelling, decreased mobility in an affected joint, muscle wasting, a limp, or a palpable soft-tissue mass. Individuals with rare bone tumors may also present with an enlarged liver or spleen. Tests: Most bone tumors can be visualized on x-rays of the affected bone or joint. Percutaneous imaging-guided needle biopsy or bone marrow aspiration may be performed to determine if the tumor is benign or malignant. If the results of biopsy and aspiration confirm malignancy, preoperative tests may include a chest x-ray, CT, and MRI, as well as routine blood tests (CBC, coagulation tests, and chemistry profile) and urinalysis to determine overall condition and to help identify if metastasis is likely from a primary malignant tumor. Additionally, a bone scan (scintigraphy) may be performed to evaluate whether a malignant tumor has spread to other areas (metastasized) of bone. |
Source: Medical Disability Advisor
| Some benign bone tumors may not require treatment, but will be assessed periodically to check for progress or regression. Other benign bone tumors may require open or radical surgery (resection). The resection of many benign bone tumors often leaves large deficits in the involved bone. Such deficits may be filled with bone grafted from the individual's own bone (autograft), with cadaveric bone (allograft), or with synthetic bone substitutes (e.g., coralline hydroxyapatite).
Advances in the treatment of malignant bone tumors include the use of multi-agent chemotherapy, making it possible to perform surgery that saves the limb from amputation (limb- salvage surgery) and offers long-term satisfactory results. The individuals being treated are usually given a course of preoperative (neoadjuvant) chemotherapy. A response to chemotherapy that results in greater than 90% cell or tissue death (necrosis) is considered a good response. Ideally, individuals with a good response to chemotherapy are considered good candidates for limb-salvage surgery. A poor response to chemotherapy may lead to amputation. Surgery is followed by a course of postoperative chemotherapy. In many instances, radiation is used in combination with chemotherapy for tumor control in the surrounding tissue. Rehabilitation and physical therapy programs are often indicated after chemotherapy, radiation, or surgery.
Treatment for metastatic malignant tumors depends upon the primary tissue or organ that has become involved. Though preoperative chemotherapy may be administered, the treatment of choice involves surgery to remove the diseased portion of the bone and reconstruction of the arm or leg. Surgery may involve a complete removal of the bone (resection) or amputation.
Another type of surgical procedure, hyperthermic isolated limb perfusion (HILP), delivers heat and chemical agents to the tumor site through the vascular system. This technique, used only on extremities, has been shown to salvage limbs and reduce complications and healing time.
A metal prosthetic device or a bone graft from a cadaver may be inserted into a weakened bone segment for strengthening of the limb. Tumor growth may be slowed with estrogen or hypothalamic hormone therapy. |
Source: Medical Disability Advisor
| Most individuals with benign bone tumors are completely cured with treatment.
The outcome for malignant tumors depends on the tumor grade, whether the tumor has invaded the center of the bone, and whether the cancerous cells have already spread to other areas (metastasized). Low-grade malignant tumors have an excellent prognosis. A small percentage of osteosarcomas, especially in young people, involve only the surface of the bone and generally are of a low histologic grade. Individuals with low-grade osteosarcomas are frequently treated with surgery only and have a better survival rate than individuals with higher-grade osteosarcomas.
Individuals with osteosarcoma, the most common form of malignant bone tumor, are rarely cured. The survival rate for high-grade osteosarcoma (with bone metastases) is under 25% (Cripe). The 5-year survival rate for nonmetastatic (low-grade) osteosarcoma is 60% to 70% (ACS). Limb-salvage surgery and amputation involve significant body disfigurement and morbidity.
For chondrosarcoma, prognostic variables include the presence of metastatic disease and a high histologic grade of the disease. Chondrosarcomas that have spread to lymph nodes and other organs are cured in only 29% of cases (Hide). The pelvis as a primary site of disease also carries a worse prognosis than peripheral sites because surgery may not be an option. However, individuals with low-grade tumors have a 90% survival rate (Hide).
For Ewing's sarcoma, the most important prognosis variable is whether the cancer has spread, as the number of metastases almost directly correlates to prognosis. Other prognostic factors include the size of the primary tumor and whether the tumor is situated in a central or peripheral location such as in the extremities. The current expected survival rate is 70% for nonmetastatic disease at the time of diagnosis (Toretsky). A less favorable cure rate of lower than 25% is expected for individuals whose cancer has spread (Toretsky). |
Source: Medical Disability Advisor
| Rehabilitation for individuals with bone tumors depends on the location of the tumor, whether the tumor is benign or malignant, and whether limb-salvage surgery or amputation was required. If surgery was required, rehabilitation may include therapy to regain range or motion, strength, and functional mobility following limb-sparing surgery, or fitting of a prosthesis and movement retraining following amputation. If the bone tumor affects a lower extremity, the physical therapist will also focus on training for proper ambulating (walking) at the appropriate time. Occupational therapy will help individuals arrange their homes and organize their lives in ways that support their physical and mental well being, and provide activities to relieve the boredom of inactivity.
Supportive rehabilitation allows the individual to gain some control over the ordinary activities of life and to cope emotionally. This may take the form of group vocational rehabilitation and may help with the individual's transition back into the workplace. Palliative rehabilitation allows individuals in advanced stages of the disease to achieve some level of physical comfort and provides emotional support and assistance in day-to-day functioning. |
Source: Medical Disability Advisor
| Some benign bone tumors may recur even after surgical removal (excision).
Complications of malignant bone tumors include muscle wasting, pathological fractures, and the risk of developing a secondary malignancy. If the bone tumor is secondary to metastatic cancer in another organ, the progressiveness (stage) of the original cancer can be a complicating factor.
Complications of primary bone cancer are related to metastasis, i.e., if a metastatic sarcoma spreads (metastasizes) to surrounding tissue, adjoining organs, other bones, or to more distant parts of the body. In Ewing's sarcoma and osteosarcoma, the most common sites of metastases are other bones and the lungs.
Complications related to treatment may include a temporary burn to the skin and fatigue from radiation therapy; nausea, vomiting, mouth sores, hair loss, and lowered resistance to infection from chemotherapy; and infection of the surgical site and possible blood clotting disturbances from surgery. Many older individuals find it difficult to tolerate surgery, chemotherapy, and radiation therapy because their overall health status is often poor. |
Source: Medical Disability Advisor
| Transfer to sedentary duties may be required for individuals who have undergone amputations. A leave of absence for surgery and postoperative therapy and rehabilitation may be required. Patients with amputation may require a prosthesis for improvement of ambulation in the workplace. |
Source: Medical Disability Advisor
| If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case. Regarding diagnosis:
- Is the tumor benign, primary, or metastatic?
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Does individual complain of pain? Does it worsen at night?
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Is swelling, stiffness, or tenderness present?
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Can individual feel a mass at the tumor site?
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Has individual had a bone fracture?
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On exam, were swelling, decreased mobility in a joint, or muscle wasting evident?
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Does individual limp?
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Does individual have an enlarged liver or spleen?
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Were x-rays, bone biopsy, and bone marrow testing done? Chest x-ray, CT, MRI, and bone scan?
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Were conditions with similar symptoms ruled out?
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Does individual have primary or secondary bone cancer?
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Is there a history of excessive exposure to radiation?
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Does individual have any inherited genetic risk factors? Additional risk factors for primary bone cancer such as Paget's disease, overactive parathyroid gland, long-term osteomyelitis, radiation and chemotherapy treatments for other conditions, or previously fractured bones? Does individual have any risk factors for secondary bone cancer such as cancer in the kidney, lung, prostate, breast, or thyroid?
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Does individual complain of pain in the bones and joints not necessarily associated with movement? Is it dull and constant or felt only when pressure is applied? Does individual complain of fever, weight loss, fatigue, impaired mobility, or spontaneous bone fractures?
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Were blood and urine tests done? Bone biopsy, bone scan?
Regarding treatment:
- If treatment is not needed, does individual have regular follow-up?
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Was surgical resection necessary?
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Was a bone graft done?
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If necessary, has individual had pre- and postoperative chemotherapy? Radiation therapy?
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Was amputation or limb-salvage surgery necessary?
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Did individual receive antibiotics, if necessary?
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If indicated, is individual receiving hormone therapy or removal of hormone-producing organs?
Regarding prognosis:
- Is individual active in rehabilitation?
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Can individual's employer accommodate necessary restrictions?
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Does individual have any conditions that may affect ability to recover?
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Has the tumor recurred?
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Has individual had any pathological fractures? Muscle wasting?
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Did a second malignancy develop?
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Have any complications developed such as metastasis or the tumor encompassing an area that confounds or does not allow for its removal?
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Does individual have any treatment complications such as a temporary burn to the skin, fatigue, nausea, vomiting, mouth sores, hair loss, lowered resistance to infection, infection of the surgical site, or possible blood clotting disturbances from the surgery?
|
Source: Medical Disability Advisor
| Cripe, Timothy P. "Osteosarcoma." eMedicine. Eds. Samuel Gross, et al. 10 Feb. 2004. Medscape. 4 May 2005 <http://emedicine.com/ped/topic1684.htm>.Toretsky, Jeffrey A. "Ewing Sarcoma and Primitive Neuroectodermal Tumors." eMedicine. Eds. Samuel Gross, et al. 17 Jan. 2005. Medscape. 4 May 2005 <http://emedicine.com/ped/topic2589.htm>. "What is Bone Cancer?" American Cancer Society. 27 May. 2005. 4 May 2005 <http://www.cancer.org/docroot/CRI/content/CRI_2_4_1X_What_Is_bone_cancer_2.asp?sitearea=CRI>. |
Source: Medical Disability Advisor
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