What is Extraskeletal Myxoid Chondrosarcoma?
Extraskeletal myxoid chondrosarcoma (EMCS) is an intermediate to low- grade tumor, characterized by a mass (lump) accompanied by the symptoms of pain and tenderness (sensitive to touch).
A slow grade tumor is an abnormal growth of the tissue, either benign or malignant, which grows and spread slowly as compared to the high-grade tumor.
EMCS Usually develops in the deep soft tissues of the upper arms, shoulders, hips, legs above the knees, and most frequently, in the soft tissues of the thighs and popliteal fossa (knee pit). The mean size of the tumor is approximately 9.3cm.
Among all the soft tissue sarcomas, the prevalence (frequency of occurrence of a disease) of EMCS is about less than 3%. It is more common in males as compared to females; with a mean age at the diagnosis of the tumor is about 50 years. It is very rare in children and teenagers.
Extraskeletal myxoid chondrosarcoma may arise both from the bones and the soft tissue, but this subtype of sarcoma is most commonly developed from soft tissues.
A five-year survival rate is up to 90%, and only in 50% of the cases, the tumor may spread to the other parts of the body with the most frequent incidence (occurrence) in the lungs.
A genetic mutation (alteration) in a certain gene is the cause behind the development of EMCS.
Signs and Symptoms
- A slowly growing palpable mass or swelling
- Sharp or dull pain at the site of swelling
- Swelling or redness of the tumor site
- Tenderness ( pain or sensitivity on touch)
- Fracture of the bone at the local site of the tumor
Diagnosis and Treatment
Following diagnostic tests and procedures can be used to make a diagnosis. These are:
- Radiological and imaging techniques like CT-scan, PET-scan, MRI, and X-ray.
- FNAC (fine needle aspiration cytology)
- Tumor Biopsy
In most of the cases, where the tumor is not spread to the other organs, surgical wide local resection of the tumor followed by chemotherapy is the most recommended treatment option.
Resection is the process of cutting out the affected part of the tissue or organ by using surgical techniques.
Other treatment options are radiotherapy and chemotherapy.
What support can we give for Extraskeletal Myxoid Chondrosarcoma?
Extraskeletal Myxoid Chondrosarcoma is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. We provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Extraskeletal Myxoid Chondrosarcoma, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
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