Chondrosarcoma

(medicine) A malignant tumor of cartilage.

Key Terms: Carcinogen, Epiphysis.

Definition

Chondrosarcoma is a malignant tumor that arises from cells that produce cartilage, the rubbery tissue around joints. Therefore, it is a type of sarcoma that is predominantly found in the area around bones.

Description

Sarcomas of the bone are rare and represent about 0.2% of all new cancer cases each year. The two most common forms of bone cancer are osteosarcoma and Ewing's sarcoma. Among the less common are chondrosarcoma, fibrosarcoma, and malignant fibrous histiocytoma, all of which arise from spindle cell neoplasms.

Chondrosarcomas arise from chondroblasts, cells that form cartilage. Cartilage is the matrix found at the tip of the nose and ears. However, cancer that develops from chondroblasts is usually observed on the surface of the pelvis, in the femur of the upper leg, around the shoulder, in the humerus of the upper arm, and in the ribs.

Depending on the type and location of the chondrosar-coma, the tumor can either be high grade and aggressive or low grade and not as invasive. There are two different categories of chondrosarcomas—classic chondrosarcomas and variant chondrosarcomas. Together they have five main types.

Central chondrosarcoma and peripheral chondrosar-coma are both classic chondrosarcomas. Central chondrosarcoma occurs within a bone, and peripheral chondrosarcoma develops on the surface of a bone. Both can develop as a primary tumor or as a secondary tumor to an existing tumor elsewhere in the body. Most, however, are primary tumors. Seventy-six percent of primary chondrosarcomas occur centrally within a bone.

There are three variant chondrosarcomas: clear cell chondrosarcoma, mesenchymal chondrosarcoma, and dedifferentiated chondrosarcoma. Clear cell chondrosar-coma is the most rare form of chondrosarcoma. It is a low grade, slow growing tumor that typically occurs locally in the epiphysis, or end part, of long tubular bones such as the femur and humerus, meaning that it does not normally invade into surrounding soft tissue. As the name implies, cells biopsied from this type of chondrosarcoma appear clear with many large vacuoles.

Mesenchymal chondrosarcoma is another rare variant. However, as opposed to clear cell chondrosarcoma, it is highly malignant and frequently metastasizes, commonly to the lungs, lymph nodes and other bones. This variant has a tendency to develop in flat bones such as vertebra, the pelvis, or the skull, as opposed to long tubular bones. Under a microscope, the cells appear round and contain spindle cell elements and neoplastic cartilage formation.

Dedifferentiated chondrosarcoma is also rare and is the most malignant form of chondrosarcoma. It is characterized by the presence of a mix of low-grade chondrosar-coma and has undergone malignant degeneration, producing a fully malignant soft tissue mass that is no longer identifiable as cartilage. These cancers occur most commonly in the flat bones of individuals over the age of sixty. Despite varied treatments, they are almost always fatal.

Due to the location of chondrosarcoma tumors, the result is often a decrease in the range of motion of limbs, especially tumors occurring on the epiphysis of bones such as those seen in clear cell chondrosarcoma.

Demographics

Although there are exceptions, chondrosarcomas occur mainly in older adults forty to sixty years old and typically occur more in men than in women. Chondrosarcomas are rarely seen in infants and children. Dedifferentiated chondrosarcomas predominantly arise in the elderly over the age of sixty, equally between males and females. Mesenchymal chondrosarcoma develops in the young adult population between the ages of twenty and forty years old, and it is slightly more common in females. Classic chondrosarcomas usually develop in people over the age of forty. However, when they occur in younger age groups, they have a propensity to be highly malignant, capable of metastasis.

Causes and Symptoms

As of 2005, there is little known about what causes chondrosarcomas. However, researchers have discovered that chondrosarcomas are sometimes associated with underlying benign bone tumors. They can also result as a side effect from previous radiation therapy for unrelated primary cancer treatment. Individuals with other bone diseases such as Maffucci's syndrome and Ollier's disease are at a higher risk for developing chondrosarcomas.

There are many symptoms associated with the onset of chondrosarcomas. They tend to develop slowly in most cases, except when the cancer is aggressive. The following is a list of the main symptoms that may present:

• pain
• swelling
• firm lump
• broken bone
• impeded normal range of motion
• urinary frequency (seen in pelvic chondrosarcomas)
• urinary obstruction (seen in pelvic chondrosarcomas)

The above symptoms are not always indicators of the presence of chondrosarcoma. Any one of these symptoms could be related to another, less serious condition. A doctor should be seen to diagnose the problem properly.

Diagnosis

In order to diagnose bone cancer, a doctor will take the patient's history and conduct a thorough physical exam. Blood tests will be performed to rule out other conditions and identify cancer markers.

The most revealing initial exam is an xray.Itcan show the location, size, and shape of the tumor. If a malignant tumor is present, the x ray will expose a soft tissue mass with ill-defined edges. This procedure takes less than an hour and can be performed in the doctor's office. Depending on the medical facilities, the results can be returned the same day after being interpreted by a physician, and perhaps a consulting oncologist and radiologist.

Once there is evidence of a tumor, one or more of several other procedures may be performed, including CT scans, MRI (magnetic resonance imaging), angiograms, and biopsy.

Treatment Team

If the patient is seeing a primary care provider, the provider may perform the initial diagnostic tests. However, in order to comprehensively diagnose and treat chondrosarcomas, the primary care provider will refer the patient to an orthopaedic oncologist (bone cancer specialist). Radiologists, pathologists and orthopaedic surgeons will also be involved to read x rays, examine tissue samples, and remove the tumor if necessary.

Many other individuals will be involved with the treatment of chondrosarcoma. For example, nurses and dieticians are available to explain side effects of treatment and offer suggestions on eating healthy meals to help fight the side effects. If a limb is totally or partially removed, a physical therapist or vocational therapist will assist the patient in learning how to use a prosthetic limb.

Clinical Staging, Treatments, and Prognosis

After the physician makes the diagnosis, it is important to determine the stage of the cancer. This will help reveal how far the cancer has progressed and how much tissue has been affected.

A new system of staging was adopted in 1980 by the Musculoskeletal Tumor Society. It is based on the fact that differing tissue types associated with the bone behave similarly when cancerous. This classification system uses grade (G), location (T), and lymph node involvement and metastasis (M).

Surgical grade (G) refers to how aggressive the cancer is. For example, G0 represents a benign tumor and G2 represents a highly aggressive tumor. The anatomical location (T) establishes whether or not the tumor is inside the bone (T1) or outside the bone (T2). If metastases are present, then the tumor is classified as M0; and if metastases are not present, the tumor is classified as M1. The following is a list of stages and their indications:

• Stage IA (G1, T1, M0): low grade within the bone, without metastasis
• Stage IB (G1, T2, M0): low grade outside the bone, without metastasis
• Stage IIA (G2, T1, M0): high grade within the bone, without metastasis
• Stage IIB (G2, T2, M0): high grade outside the bone, without metastasis
• Stage IIIA (G1 or G2, T1, M1): inside the bone, with metastasis
• Stage IIIB (G1 or G2, T2, M1): outside the bone, with metastasis

Physicians can employ several courses of treatment to remove chondrosarcomas. The most effective treatment is surgical removal. When performing the surgery, the doctor will remove the tumor and some healthy tissue or bone around it to ensure that the tumor does not recur near the original site. The physician may replace the removed bone with a metal device. In children, the metal device can be lengthened as the child grows, but this will require further surgeries. The fact that most chondrosarcomas tend to be low grade and slow-progressing makes this procedure one that does not necessitate entire limb removal except in extreme cases when the tumor is large.

Even individuals with low-grade chondrosarcoma that have undergone surgery experience a moderate risk of local recurrence. To combat recurrence, chemotherapy (the use of one or more cancer killing drugs) and radiation therapy (the use of high energy rays) have also been used to complement surgery. Employing chemotherapy or radiation therapy individually (without surgery) is much less effective. In fact, chondrosarcomas are generally resistant to chemotherapy alone.

Low stage chondrosarcomas (Stages IA and IB) have greater one and five-year survival rates than the high stages (Stages IIIA and IIIB). High-grade tumors are more aggressive and highly metastatic than lower grade tumors, and therefore they have a lower survival rate. Not only is the grade of the tumor (the estimate of its aggressiveness) important in determining prognosis, but the age of the patient is also crucial. Generally, chondrosarcomas that occur in childhood and infancy have a higher mortality rate than those that occur in adults.

Metastases appear later in the development of chondrosarcomas. The lungs are the sites of primary metastasis. Once metastasis to the lungs has occurred, survival rate decreases.

Coping With Cancer Treatment

Chemotherapy often results in several side effects, depending on the drug used and the patient's individual tolerance. Patients may have to deal with nausea, vomiting, loss of appetite, and hair loss. Often, chemotherapy and radiation therapy are better handled if the patient is eating well. Nurses and dieticians can aid in choosing healthful foods to incorporate into the patient's diet.

If the chondrosarcoma necessitates limb amputation, the patient will need to learn how to cope with a prosthetic device. Both physical and vocational therapists can help the patient adjust and learn to use prosthetic devices to perform daily activities in new ways.

Clinical Trials

Since chondrosarcomas are rare forms of cancer, there is still much to be learned. Clearly, surgery is the most effective treatment. New techniques in cryosurgery are being developed in various institutions across the country.

Chemotherapy trials have shown improved results with more intense regimens. Such drugs that are under study include methotrexate, leucovorin, vincristine, bacillus Calmette Guérin, doxorubicin, or a combination of two or three of these.

Patients should consult with their physicians or contact the American Cancer Society to learn what procedures are currently in clinical trials. In some cases, insurance companies will not cover clinical trial procedures. Patients should talk with their doctor and insurance company to determine which procedures are covered.

Prevention

Since little is known about what causes chondrosarcomas, there is also little known about how to prevent them. In general, the prevention of cancer can be assisted by avoiding known chemical carcinogens such as alpha-naphthylamine, carbon tetrachloride, and benzene. Another way to avoid developing cancer—especially bone cancer—is to minimize exposure to penetrating radiation such as x rays and radioactive elements. Medical x rays revolutionized the field of medicine and are used to detect and treat many diseases. In most cases, the benefits of medical x rays out-weigh the risks.

Special Concerns

Cancer treatments, especially surgical amputation, can take a physical and psychological toll on cancer patients and their families. To deal with the psychological impact, many different support groups and psychotherapists are available to help. Some therapists will consider amputation a post-traumatic stress disorder and treat it accordingly. Faith practices are also beneficial for cancer patients in dealing with their condition. Patients should discuss all options with their physician to determine what is available to them.

Questions to Ask the Doctor

• What diagnostic procedures are best for the location and type of tumor suspected?
• What treatments are best for the location and type of tumor suspected?
• What kinds of side effects will this course of treatment result?
• Are there support services available?
• What treatments are currently in clinical trials?
• What treatments will my health care insurance cover?

Once the cancer has been treated, patients should make sure to schedule follow-up appointments with their physicians. Physicians will want to monitor the patient for side effects or possible recurrence that may develop years after treatment.

Resources

Books

Rosen, Gerald, M.D. "Neoplasms of the Bone and Soft Tissue." In Cancer Medicine, edited by Robert C. Bast, Jr., M.D., et al. London: BC Decker, Inc., 2000, pp.1870-95.

Periodicals

Lee, Francis, M.D., et al. "Chondrosarcoma of Bone: An Assessment of Outcome." The Journal of Bone and Joint Surgery March 1999: 326-38.

Mitchell, A.D., et al. "Experience in the Treatment of Dedifferentiated Chondrosarcoma." The Journal of Bone and Joint Surgery January 2000: 55-61.

Organizations

American Cancer Society. .

National Cancer Institute. .

—Sally C. McFarlane-Parrott

A malignant neoplasm composed of cartilage-like tissue.

Chondrosarcoma. (Sapp/Eversole/Wysocki, 2004)

A malignant tumor derived from cartilage cells or their precursors.

This article's tone or style may not be appropriate for Wikipedia. Specific concerns may be found on the talk page. See Wikipedia's guide to writing better articles for suggestions. (December 2007)

Chondrosarcoma
Classification and external resources
Histopathogic image of chodrosarcoma of the chest wall. Surgical resection of recurrent mass. H & E stain.
ICD-9	170.9
ICD-O:	M9220/3-9240/3
MeSH	[1]

A chondrosarcoma is a type of cancer^[1] of the cartilage. Chondrosarcoma is a cartilage-based tumor and is in a category of cancers called sarcomas. About 25% of primary bone cancers (meaning those which start in the bone) are chondrosarcomas. This disease can affect people or animals of any age, although it is more common among older people than among children.^[2]

Contents 1 Classification 2 Diagnosis 3 Causes 4 Treatment 5 Prognosis 5.1 Chondrosarcoma 6 References 7 External links

Classification

Physicians grade chondrosarcomas based chiefly on how fast they grow and the likelihood of metastasis. Grade 1 is the slowest growing form. Grades 2 and 3 are faster-growing cancers, and some medical facilities also assign grade 4 to the fastest growing and usually metastasized occurrences.

The most common sites for chondrosarcoma to grow are the pelvic and shoulder bones along with the superior metaphysial and diaphysial regions of the arms and legs.^[3] But chondrosarcoma may occur in any bone, even the base of the skull.

ICD-O codes are used for more precise classification:

• 9220 = Chondrosarcoma NOS
• 9221 = Juxtacortical chondrosarcoma
• 9231 = Myxoid chondrosarcoma
• 9240 = Mesenchymal chondrosarcoma
• 9242 = Clear cell chondrosarcoma
• 9243 = Dedifferent chondrosarcoma

Diagnosis

Like all other bone tumors and disease processes, diagnosis is made primarily by imaging. There are no blood tests to determine a diagnosis of chondrosarcoma. The most characteristic imaging findings are on CT, where detection of a chondroid matrix is present.

Nearly all chondrosarcoma patients appear to be in good health. This form of cancer generally doesn't affect the whole body. Often, patients are not aware of the growing tumor until there is a noticeable lump or pain. Earlier diagnosis is generally accidental, when a patient undergoes testing for another problem and physicians discover the cancer. Occasionally the first symptom will be a broken bone at the cancerous site. Any broken bone that occurs from mild trauma warrants further investigation, although there are many conditions that can lead to weak bones, and this form of cancer is not a common cause of such breaks.^[4]

Causes

The cause is unknown. Patients may have a history of enchondroma or osteochondroma.

Treatment

Treatment depends on the location of the disease and the aggressiveness of the tumors.^[3] Because chondrosarcomas are rare, they are treated at specialist hospitals with Sarcoma Centers.

Surgery is the main form of treatment for chondrosarcoma. Musculoskeletal tumor specialists or orthopedic oncologists are usually chosen to treat chondrosarcoma, unless it is located in the skull, spine, or chest cavity, in which case, a neurosurgeon or thoracic surgeon experienced with sarcomas is chosen. Often, a limb-sparing operation can be performed, however in some cases amputation is unavoidable. Amputation of the arm, leg, jaw, or half of the pelvis (called a hemipelvectomy) may be necessary in some cases.

Chemotherapy or traditional radiotherapy are not very effective for most chondrosarcomas, although proton therapy is showing promise with local tumor control at over 80%.^[5]

Complete surgical ablation is the most effective treatment, but sometimes this is difficult. Proton therapy Radiation can be useful in awkward locations to make surgery more effective.

Prognosis

Prognosis depends on how early the cancer is discovered and treated. For the least aggressive grade, about 90% of patients survive more than five years after diagnosis. People usually have a good survival rate at the low grade volume of cancer.^[2] For the most aggressive grade, more than a quarter of patients live more than five years.

Tumors may recur in the future. Follow up scans are extremely important for chondrosarcoma to make sure there has been no recurrence or metastasis, which usually occurs in the lungs.

Chondrosarcoma

References

1. ^ Gelderblom H, Hogendoorn PC, Dijkstra SD, et al. (March 2008). "The clinical approach towards chondrosarcoma". Oncologist 13 (3): 320–9. doi:10.1634/theoncologist.2007-0237. PMID 18378543. http://theoncologist.alphamedpress.org/cgi/pmidlookup?view=long&pmid=18378543. 
2. ^ ^{a b} "Chondrosarcoma: Overview - eMedicine Radiology". http://www.emedicine.com/radio/topic168.htm. Retrieved 2009-02-14. 
3. ^ ^{a b} "Chondrosarcoma : Cancerbackup". http://www.cancerbacup.org.uk/Cancertype/Bone/Typesofbonecancer/Chondrosarcoma#3340. Retrieved 2009-02-14. 
4. ^ "Caring Medical - Symptoms - Chondrosarcoma". http://www.caringmedical.com/conditions/Chondrosarcoma.htm. Retrieved 2009-02-14. 
5. ^ "Proton Therapy at PSI (Protonentherapie)". http://p-therapie.web.psi.ch/e/klinikerfahrung.html. Retrieved 2009-02-14. 

External links

• Chondrosarcoma of Bone
• CancerBACUP Chondrosarcoma Info Page
• Bone Tumor.org Fact Page on Chondrosarcoma
• What is Sarcoma from the Sarcoma Alliance

v • d • e Connective tissue neoplasm: Osseous and Chondromatous tumors (ICD-O 9180-9269) (C40-C41/D16, 170/213) Diaphysis Myeloid Multiple myeloma Epithelial Adamantinoma PNET/Ewing family Ewing's sarcoma Metaphysis Osteoblast Osteoid osteoma · Osteoblastoma Osteoma/osteosarcoma Chondroblast Chondroma/ecchondroma/enchondroma (Enchondromatosis) · Chondrosarcoma (Mesenchymal chondrosarcoma, Myxoid chondrosarcoma) Osteochondroma (Osteochondromatosis) Chondromyxoid fibroma Fibrous Ossifying fibroma · Fibrosarcoma Epiphysis Chondroblast Chondroblastoma Myeloid Giant cell tumor of bone Other/ungrouped Notochord Chordoma bone and cartilage navs: anat/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous signs, proc

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