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Extraskeletal Myxoid Chondrosarcoma (EMC)

Last updated Sept. 11, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Extraskeletal Myxoid Chondrosarcoma (EMC) is an infrequent, low-grade tumor of the soft tissues, forming around bones. Middle-aged to elderly individuals are most prone to this tumor formation.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Chordoid Sarcoma
  • EMC (Extraskeletal Myxoid Chondrosarcoma)

What is Extraskeletal Myxoid Chondrosarcoma? (Definition/Background Information)

  • Extraskeletal Myxoid Chondrosarcoma (EMC) is an infrequent, low-grade tumor of the soft tissues forming around the bones. Middle-aged to elderly individuals are most prone to this tumor formation
  • Most of these tumors form deep within the body tissues, though a few have been found beneath the skin surface too. The most common location for this tumor is the thigh, followed by the knee, buttocks, and trunk
  • Extraskeletal Myxoid Chondrosarcoma tumors can grow to large sizes and compress other tissues and organs causing restricted range of motion, sensation of pain, etc. A diagnosis of EMC may be confirmed through a tissue biopsy
  • Any combination of chemotherapy, radiation therapy, and invasive procedures, may be used to treat the tumor. However, the prognosis for EMC is guarded

Who gets Extraskeletal Myxoid Chondrosarcoma? (Age and Sex Distribution)

  • Extraskeletal Myxoid Chondrosarcomas are mostly observed during the mid-to-late adult phase of an individual (age 40-60 years)
  • Much younger adults are also known to be occasionally affected; however, EMC is rare in children and adolescents
  • There is a slight predisposition towards the male sex (with a male-to-female ratio of 2:1)
  • There is no known ethnic or racial preference

What are the Risk Factors for Extraskeletal Myxoid Chondrosarcoma? (Predisposing Factors)

  • The risk factors for Extraskeletal Myxoid Chondrosarcoma development are not clearly identified, but it is thought to be associated with certain genetic defects

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Extraskeletal Myxoid Chondrosarcoma? (Etiology)

The exact cause and mechanism of Extraskeletal Myxoid Chondrosarcoma formation is unknown.

  • The tumor is thought to occur as a consequence of genetic defects that take place due to certain irregular chromosomal exchanges
  • Genetic defects caused by gene translocation have been observed on many chromosomes in the tumor cells

What are the Signs and Symptoms of Extraskeletal Myxoid Chondrosarcoma?

The presentations are based on the location of the tumor. Extraskeletal Myxoid Chondrosarcoma signs and symptoms include:

  • In the initial growing phase of the tumors, they are normally asymptomatic
  • The thigh, knee, buttock, and upper extremities, account for up to 80% of all EMC occurrences. This is followed by the torso, chest, breast, and abdominal cavity. All tumors are observed around the bones
  • The soft tissue tumors grow at a moderate rate, and then suddenly start rapidly progressing in size
  • Due to large size of the tumor, the adjoining organs, nerves, and muscles, may be compressed or restricted, which may cause a constraint in the range of motion of the affected limb. Often these signs along with pain and tenderness, are the first indications of the tumor
  • Lesions beneath the skin (in certain rare cases), may appear as painful inflammations

How is Extraskeletal Myxoid Chondrosarcoma Diagnosed?

A diagnosis of Extraskeletal Myxoid Chondrosarcoma may be made using the following tools:

  • Physical examination, evaluation of patient’s medical history
  • Histopathological studies conducted on a biopsy specimen - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis. A high cell activity and profuse hemorrhaging is observed with this tumor type
  • CT scan, MRI scan of the affected region
  • A differential diagnosis may be necessary to eliminate other similar types of sarcomas

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Extraskeletal Myxoid Chondrosarcoma?

Complications are dependent on the site and severity of Extraskeletal Myxoid Chondrosarcoma. These include:

  • Bone damage that may also lead to limb amputations
  • Lung and lymph node metastasis
  • Deep-seated tumors (those buried in the body tissues), may create problems for adjoining tissues and organs by compressing them (due to the mass effect of the tumor)
  • Blood loss during invasive treatment methods may be heavy; blood may accumulate outside the blood vessels
  • Damage to vital nerves, blood vessels, and surrounding structures during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy

How is Extraskeletal Myxoid Chondrosarcoma Treated?

Treatment measures for Extraskeletal Myxoid Chondrosarcoma include the following:

  • A combination of chemotherapy, radiation therapy, and invasive procedures, are used to treat EMC
  • Wide surgical excision with removal of the entire lesion is the standard and preferred treatment mode. If the tumor is not fully removed, then it might recur
  • Radiation therapy may be administered, before or after the surgical procedure
  • Vascular embolization of the tumor, by blocking the blood vessels feeding the tumor, is used to provide temporary relief from the symptoms and reduce blood loss, during a surgical procedure
  • In case the tumor has metastasized into the lymph nodes, a surgery may be then followed by chemotherapy
  • When EMC is at an inaccessible location, or is unsafe for surgical intervention; non-invasive procedures are adopted
  • Post-operative care is important: A minimum activity level is to be ensured until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Extraskeletal Myxoid Chondrosarcoma be Prevented?

  • Current medical research has not established a way of preventing Extraskeletal Myxoid Chondrosarcoma
  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations, are mandatory for those who have been diagnosed with the tumor
  • Due to both its metastasizing potential and chances of recurrence, often several years of active follow-up vigilance is necessary

What is the Prognosis of Extraskeletal Myxoid Chondrosarcoma? (Outcomes/Resolutions)

  • Extraskeletal Myxoid Chondrosarcomas are rare, but malignant tumors that are not yet completely understood
  • The long-term prognosis depends on a combination of factors, such as:
    • Age of the individual
    • Tumor size and stage at detection
    • Type and location of the tumor
    • Ki-67 value - a protein found in cells that is a good indicator of cell activity
    • Its response to treatment and medical therapy
  • Elderly males with tumor size over 5 cm, with local recurrence, and a high cell activity indicated by the biopsy specimen, are all factors that point to a poor outcome
  • The long-term outcome of EMC, over a 5-year period is a 37-87% mortality rate. This value is generally on the lines of other sarcoma type outcomes

Additional and Relevant Useful Information for Extraskeletal Myxoid Chondrosarcoma:

  • Chondrosarcoma is a class of soft tissue and bone tumor, belonging to the sarcoma family. These develop from cells that were originally meant to form cartilages, but get transformed due to various reasons into cancerous tumors
  • The Extraskeletal Myxoid Chondrosarcoma tumor cells are primarily composed of premature, oval or spindle shaped, chondroblasitc cells and form as well-defined masses

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 29, 2014
Last updated: Sept. 11, 2018