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Mesenchymal Chondrosarcoma

Last updated Nov. 20, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Mesenchymal Chondrosarcoma (MCS) is a rare and aggressive malignancy that in many cases affect the bones, and in some cases, the soft tissues of the body.

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

  • MCS (Mesenchymal Chondrosarcoma)

What is Mesenchymal Chondrosarcoma? (Definition/Background Information)

  • Mesenchymal Chondrosarcoma (MCS) is a rare and aggressive malignancy that in many cases affect the bones, and in some cases, the soft tissues of the body
  • In general, chondrosarcomas are malignant tumors that mostly arise in the bone. Mesenchymal Chondrosarcomas constitute about 5% of all chondrosarcomas. However, they grow faster, and are hence, more aggressive than all other chondrosarcoma types
  • They are subdivided into the following 2 types:
    • Skeletal Mesenchymal Chondrosarcoma (2/3rd of the cases) that involve the bones
    • Extraskeletal Mesenchymal Chondrosarcoma (1/3rd of the cases) that involve the soft tissues of the body
  • The cause of tumor formation is reportedly due to genetic mutations and chromosomal aberrations. The risk factors for Mesenchymal Chondrosarcoma remain presently unidentified
  • Teens and young adults are most prone to Mesenchymal Chondrosarcoma. The signs and symptoms depend upon the location and the size of the tumor. MCS tumors can grow to large sizes and compress other tissues and organs
  • Any combination of chemotherapy, radiation therapy, and surgical measures may be used to treat Mesenchymal Chondrosarcoma. However, the prognosis for MCS is generally poor

Who gets Mesenchymal Chondrosarcoma? (Age and Sex Distribution)

  • Mesenchymal Chondrosarcomas are uncommon tumors that are mostly observed in adolescent children and young adults in the 10-30 year’ age group
  • There is a slight predisposition towards the female gender, though both males and females are affected
  • There is no known ethnic or racial preference

What are the Risk Factors for Mesenchymal Chondrosarcoma? (Predisposing Factors)

  • Currently, the specific risk factors for Mesenchymal Chondrosarcoma development are unknown

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 Mesenchymal Chondrosarcoma? (Etiology)

The cause of Mesenchymal Chondrosarcoma formation is due to genetic factors. Currently, studies indicate the following defects:

  • Involvement of HEY1 and NCOA2 genes, which is known to result in abnormalities (deletions) on chromosome 8

What are the Signs and Symptoms of Mesenchymal Chondrosarcoma?

Mesenchymal Chondrosarcoma signs and symptoms may include:

  • The presentations are based on the location of the tumor:
    • Skeletal tumors may affect any bone in the body; common locations include the spine, ribs, jaw, and thighbone (femur)
    • Extraskeletal tumors affect the soft tissues of the arms and legs and head and neck region; but any part of the body may be involved
  • In the initial growing phase of the tumors, they may be asymptomatic
  • MCS tumors may form a fast-growing mass
  • Large-sized tumor may compress the adjoining organs, nerves, and muscles (due to mass effect of the tumor)
  • There may be pain and tenderness (in lesions beneath the skin)

How is Mesenchymal Chondrosarcoma Diagnosed?

A diagnosis of Mesenchymal Chondrosarcoma may involve the following tools:

  • Complete physical examination and comprehensive evaluation of the individual’s medical history
  • X-ray studies of the affected region
  • CT, MRI scan of the affected region

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include:

  • Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy may be recommended.
  • Core biopsy of the tumor
  • Open biopsy of the tumor

Tissue biopsy:

  • A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

A differential diagnosis to eliminate the following tumors is often considered, before arriving at a definitive diagnosis:

  • Conventional chondrosarcoma
  • Osteosarcoma
  • Chondroblastoma

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 Mesenchymal Chondrosarcoma?

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

  • Deep-seated tumors (those buried in the body tissues) may affect the adjoining tissues and organs by compressing them. Large tumors can lead to organ failure
  • Tumors affecting the bones may cause its weakening, and hence, be prone to easy fractures
  • Metastasis to local and regional sites
  • Tumor recurrence following its surgical removal
  • Damage to vital nerves, blood vessels, and surrounding structures during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy

How is Mesenchymal Chondrosarcoma Treated?

A combination of chemotherapy, radiation therapy, and invasive procedures, are used to treat Mesenchymal Chondrosarcoma. The treatment is addressed on an individual case-by-case basis.

Treatment measures for MCS may include the following:

  • Wide surgical excision with removal of the entire lesion is the standard and preferred treatment mode
  • Radiation therapy and chemotherapy may be administered, before or after the surgical procedure
  • When MCS 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 Mesenchymal Chondrosarcoma be Prevented?

  • Current medical research has not established a method of preventing Mesenchymal Chondrosarcoma.
  • 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 Mesenchymal Chondrosarcoma? (Outcomes/Resolutions)

  • Mesenchymal Chondrosarcomas are malignant and aggressive tumors. The prognosis of individuals with the tumor is mostly poor. The tumor is known to cause widespread metastasis
  • In general, the prognosis depends on a combination of factors, such as:
    • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
    • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
    • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
    • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
    • Individuals with bulky disease have a poorer prognosis
    • Involvement of the lymph node can adversely affect the prognosis
    • Involvement of vital organs may complicate the condition
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
    • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
    • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
    • Progression of the condition makes the outcome worse
  • An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment

Additional and Relevant Useful Information for Mesenchymal 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.

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: March 28, 2017
Last updated: Nov. 20, 2018