What is Extra Skeletal Ewing Sarcoma?
Extra Skeletal Ewing sarcoma is a rare, cancerous growth of rapidly multiplying nature that develops in the soft tissues instead of bones. It is high a grade tumor with poor differentiation from other soft tissue tumors. High-grade tumors are those who tend to grow and spread more quickly than low-grade tumors. Most frequently, they are developed from the soft tissues of the chest wall (ribs and shoulder blades), paravertebral region (anterior, posterior and lateral region of the vertebral column), abdomen, pelvis, gluteal region (buttocks), and lower extremities (part of the body from the hip to the toe).
It is more common in children and teenagers. The annual incidence rate of extra skeletal Ewing sarcoma is about 1-3 cases per 1 million population. It is more common in males as compared to females.
Extra skeletal Ewing sarcoma (ESES) has no clear, exact known cause. Some genetic mutations in chromosome number 22 and 11 are considered responsible for the development of ESES.
Signs and Symptoms
The patient can present with the following signs and symptoms. These are:
- A large and palpable mass.
- A mass or lump that is usually painless, it is painful only in 30% cases.
- A mass or lump most commonly present in the soft tissues of the thorax and lower extremities.
Patients who have distant metastasis of the disease may also suffer from the following signs and symptoms. These are;
- Night sweats
- Weight loss
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, a surgical wide local resection of the tumor followed by radiotherapy 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 multi-drug chemotherapy.
It has a high incidence (frequency of diseases) of metastasis (spreading to the other parts of the body like into the lungs and bones) with a 5- year survival rate for a patient who presents with a localized tumor is 50%. The best prognosis was observed in patients under the age of 16 who had undergone surgical resection and chemotherapy.
What support can we give for Extra skeletal Ewing Sarcoma?
Extra skeletal Ewing Sarcoma 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 Extra skeletal Ewing Sarcoma, 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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