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Mesenchymal Chondrosarcoma

NORD is very grateful to Robert G. Maki, MD, PhD, Departments of Medicine, Pediatrics, and Orthopaedics, Mount Sinai School of Medicine, New York, NY, for assistance in the preparation of this report.

Synonyms of Mesenchymal Chondrosarcoma

  • No synonyms found.

Disorder Subdivisions

  • Extraskeletal Mesenchymal Chondrosarcoma

General Discussion

Mesenchymal chondrosarcoma is an extremely rare, often aggressive form of cancer. It is a variant type of conventional chondrosarcoma. Conventional chondrosarcoma is a form of bone cancer that arises from cartilage cells. Cartilage is the specialized tissue that serves as a buffer or cushion at joints. Most of the skeleton of an embryo consists of cartilage, which is slowly converted into bone. Approximately two-thirds of cases of mesenchymal chondrosarcoma affect the bones, especially the spine, ribs or jaws. The remaining cases occur in areas of the body other than bone (extraskeletal, that is, occurring in soft tissues like muscle and fat). Unlike conventional chondrosarcomas, mesenchymal chondrosarcomas occur with greater frequency in young adults. This form of cancer often spreads (metastasizes) to other areas of the body and can cause life-threatening complications.

Symptoms

The symptoms of mesenchymal chondrosarcoma may vary depending upon the exact location and progression of the tumor. Most mesenchymal chondrosarcomas arise in the bones of the jaw, spine or the ribs. However, the tumor can arise almost anywhere within the body. The arms and legs are also frequent sites of tumor development, especially the main bone of the thigh (femur). Approximately one-third of cases occur outside of bone, appearing as soft tissue masses. Cases that occur outside of bone may be referred to as extraskeletal mesenchymal chondrosarcoma and often affect the central nervous system or muscle. The membranes that surround the brain and spinal cord (meninges) are a frequently affected area.

The specific symptoms associated with mesenchymal chondrosarcoma may be vague and nonspecific. Many individuals may develop pain and swelling in the affected area. Such vague symptoms can be present for a long time before a diagnosis is made. If a tumor grows large enough it can compress nearby structures and cause additional symptoms. For example, a tumor that compresses the spinal cord can cause paralysis. A mesenchymal chondrosarcoma in the eye socket (orbit) can cause pain, swelling, visual disturbances and protrusion of the eyeball (exophthalmos).

Mesenchymal chondrosarcomas are an aggressive form of cancer that can spread (metastasize) to other areas of the body, especially the lungs, liver, lymph nodes and other bones and may cause life-threatening complications.

Causes

The exact cause of mesenchymal chondrosarcoma is unknown. Investigators are conducting ongoing basic research to learn more about the many factors that may result in cancer. No identifiable risk factors for the development of mesenchymal chondrosarcoma have been discovered.

In individuals with cancer, including mesenchymal chondrosarcoma, malignancies may develop due to abnormal changes in the structure and orientation of certain cells known as oncogenes or tumor suppressor genes. Oncogenes control cell growth; tumor suppressor genes control cell division and ensure that cells die at the proper time. The specific cause of changes to these genes is unknown. However, current research suggests that abnormalities of DNA (deoxyribonucleic acid), which is the carrier of the body's genetic code, are the underlying basis of cellular malignant transformation. These abnormal genetic changes may occur spontaneously for unknown reasons or, more rarely, may be inherited.

Mesenchymal chondrosarcomas are thought to arise from early, immature cartilage cells called chondroblasts. Chondroblasts eventually develop into chondrocytes, the main cell that makes up cartilage.

Affected Populations

Mesenchymal chondrosarcoma was first described in the medical literature in 1959. It is an extremely rare form of cancer; fewer than 500 cancers have been reported in the medical literature as of June 2011. This tumor is most common in children and young adults (aged 10-30), but can affect individuals of any age including young children and older adults. Females are affected slightly more often than males. Mesenchymal chondrosarcoma is more likely to occur outside the bone in young adults or children and to affect bone in older adults. Mesenchymal chondrosarcomas account for approximately 1 percent of all cases of chondrosarcoma.

Related Disorders

Symptoms of the following disorders can be similar to those of mesenchymal chondrosarcoma. Comparisons may be useful for a differential diagnosis.

Extraskeletal myxoid chondrosarcoma is a tumor of soft tissue with relatively slow growth and high propensity for both local recurrence as well as innumerable metastatic sites in the lungs, a diagnosis that is less chemotherapy sensitive than mesenchymal chondrosarcoma.

Ewing sarcoma is a rare bone tumor that occurs most often in adolescents. It may also arise outside of the bone in soft tissue (extraosseous Ewing sarcoma). Ewing sarcoma is related to another type of tumor known as primitive neuroectodermal tumor (PNET). Researchers have learned that both of these tumors arise from the same primitive cell and now refer to these tumors as the Ewing family of tumors (EFT). This general term encompasses Ewing's sarcoma of bone, extraosseous Ewing's sarcoma, primitive neuroectodermal tumor, and Askin tumor (a tumor of the chest wall). Ewing sarcoma of bone accounts for approximately 87 percent of the tumors in this family. Ewing sarcoma of bone most often affects the long bone of the legs (femur) and flat bones such as those found in the pelvis and chest well. Ewing sarcoma is an aggressive cancer that may spread (metastasize) to the lungs, other bones, and bone marrow potentially causing life-threatening complications. The exact cause of these tumors is unknown. (For more information on this disorder, choose "Ewing sarcoma" as your search term in the Rare Disease Database.)

Osteosarcoma is a tumor affecting the bones. It is the most common form of bone cancer. Approximately 60 percent of cases occur in children and adolescents during the second decade of life. Osteosarcomas affect males twice as often as females. The bones most commonly affected are the long bones of the arms and legs. Symptoms may vary depending upon the location and extent of the disease. Pain, swelling, tenderness and eventually the formation of a lump may occur in the affected area. General symptoms may include fever, weight loss, anemia, and lack of energy. Osteosarcomas may weaken the surrounding bone resulting in fractures. Osteosarcomas may spread (metastasize) to other areas of the body. The exact cause of osteosarcoma is unknown. (For more information on this disorder, choose "osteosarcoma" as your search term in the Rare Disease Database.)

Additional tumors must also be differentiated from mesenchymal chondrosarcoma including osteochondromas, primary tumors of bone. (For more information on these tumors, choose the specific tumor name as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
A diagnosis of mesenchymal chondrosarcoma may be difficult. Some individuals may seek attention because of pain and swelling. A diagnosis may be made based upon a thorough clinical examination, a detailed patient history and a variety of specialized tests including x-ray studies and a procedure in which a small sample of tissue is removed and studied under a microscope (biopsy). A biopsy of a tumor or mass determines what type of cancer is present by identifying the cell type that makes up the tumor. In individuals suspected of a mesenchymal chondrosarcoma an open biopsy or a needle-guided biopsy is recommended.

Specialized imaging techniques may be used to help evaluate the size, placement, and extension of the tumor and to serve as an aid for future surgical procedures, among individuals with mesenchymal chondrosarcoma. Such imaging techniques may include computerized tomography (CT) scanning and magnetic resonance imaging (MRI). During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures. Because of the risk of a mesenchymal chondrosarcoma spreading the lungs, a CT scan of the lungs is often performed. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. Bone scans, tests that can detect areas of increased or decreased bone activity, may be performed to determine whether a mesenchymal chondrosarcoma has spread to affect other bones.

Treatment
There are implications for future treatment even in terms of placement of a biopsy incision. As a result, treatment by a multidisciplinary group of health professionals even at the time of suspicion of a bone sarcoma his highly recommended to optimize treatment. The therapeutic management of individuals with a mesenchymal chondrosarcoma typically requires the coordinated efforts of a team of medical professionals, such as physicians who specialize in the diagnosis and treatment of cancer (pediatric or medical oncologists), specialists in the use of radiation to treat cancer (radiation oncologists), surgeons, oncology nurses, and other specialists.

Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as primary tumor location, extent of the primary tumor (stage); whether the tumor has spread to distant sites; an individual's age and general health; and/or other elements. Decisions concerning the use of particular interventions should be made by physicians and other members of the health care team in careful consultation with the patient, based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks; patient preference; and other appropriate factors.

After a carefully placed biopsy to determine the diagnosis, the primary initial form of treatment for a mesenchymal chondrosarcoma is often chemotherapy, followed by surgical removal of the entire tumor and any affected tissue. Radiation may or may not be employed in the treatment of mesenchymal chondrosarcoma, depending on the extent of disease and anatomical location of the tumor. Some researchers have advocated a treatment regimen similar to that of a related bone cancer called Ewing's sarcoma.

Because mesenchymal chondrosarcomas can recur even after optimal therapy, individuals should be examined routinely after surgery (follow-up care). The specifics of follow-up care are different for each person. In some cases, the return of cancer (relapse) has not occurred until many years after treatment.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Mesenchymal Chondrosarcoma Resources

Please note that some of these organizations mat provide information concerning certain conditions potentially associated with this disorder.

NORD Member Organizations:

(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at solivo@rarediseases.org.)

Other Organizations:

References

TEXTBOOKS
De Vita Jr VT, Lawrence TS, Rosenburg SA. Eds. Cancer: Principles and Practice on Oncology. 8th ed. J.B. Lippincott Company. Philadelphia, PA; 2008:1795-1833.

Raghaven D, Brecher ML, Johnson DH. Textbook of Uncommon Cancer. 2nd ed. John Wiley & Sons. New York, NY; 1999:295.

JOURNAL ARTICLES
Dantonello TM, Int-Ween C, Leuschner I, et al. Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents and young adults. Experience of the CWS and COSS Study Groups. Cancer. 2008;112:2424-2431.

Trembath DG, Dash R, Major NM, Dodd LG. Cytopathology of mesenchymal chondrosarcomas. A report and comparison of four patients. Cancer. 2003;99:211-216.

Vencio EF, Reeve CM, Unni KK, Nascimento AG. Mesenchymal chondrosarcoma of the jaw bones. Clinicopathologic study of 19 cases. Cancer. 1998;82:2350-2355.

Nakashima Y, Unni KK, Shives TC, Swee RG, Dahlin DC. Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases. Cancer. 1986;57:2444-2453.

FROM THE INTERNET
Goldberg JM, Grier H. Mesenchymal Chondrosarcoma. The Libby Shriver Sarcoma Initiative. 2004. Available at: http://sarcomahelp.org/learning_center/mesenchymal_chondrosarcoma.html Accessed On: December 26, 2011.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2012/01/03 00:00:00 GMT+0

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