21 Feb 2024

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Ewing’s Sarcoma- Types & symptoms
Health

Ewing’s Sarcoma- Types & symptoms 

Ewing’s sarcoma is a malignant tumour of bone with neuroectodermal differentiation. These tumours are considered to be tumours of unknown regions. These tumours are highly aggressive in nature. It is also known as endothelial myeloma or round cell sarcoma. It is called round cell sarcoma because it is characterised by primitive round cells. It is the second most common sarcoma in children. It is seen that approximately 80% of the patients are below the age of 20 years. Not only that, boys are more affected than the girls. 

Blacks and Asians are rarely affected as compared to whites. Ewing sarcoma usually arises in the diaphysis of the long bones especially in the femur and the flat bones of the pelvis. But it also occurs in the tibia and spine. The shaft of the fibula is a special place for Ewing’s Sarcoma. The x-ray usually shows a moth-eaten appearance. The tumour has an onion skin or sunburst appearance on x-ray due to periosteal reaction. 

The tumour is formed by small, round, blue cells with prominent nuclei and minimal cytoplasm. The cells mostly arrange themselves in pseudo-rosette fashion. As far as the pathogenesis is concerned they are formed by translocation between chromosome no. 22 and chromosome no. 11. This translocation results in the formation of a fusion gene product i.e. EWSR1-FLI1. If treated early can be cured. 

Types of Ewing’s Sarcoma:

  1. Classical Ewing sarcoma of bone
  2. Extra osseous Ewing sarcoma
  3. Primitive neuroectodermal tumour 
  4. Askin tumour 

The genetic causes of all the above mentioned tumours are similar and therefore they are considered to be related. The only point of difference among them is the tissue in which they grow. 

The tumour growing in femurs, ribs, pelvis or shoulder blades is Ewing sarcoma of bone. In case of Extra osseous Ewing sarcoma the tumour grows in the soft tissue surrounding the bone like cartilage. Primitive neuroectodermal tumours can be found in multiple body parts because they grow in nerve tissue. Askin tumour is a kind of primitive neuroectodermal tumour that occurs in the chest. 

Diagnosis-

The diagnosis of Ewing sarcoma starts with physical examination on priority basis so as to understand the symptoms better which in turn helps the doctor to choose the best suitable treatment for the patient. Based on the deductions made by the doctors further recommend other tests and procedures. 

  1. Imaging tests- Through imaging tests the doctors can inspect the bone symptom, the appearance of the tumour, the extent of it’s growth on the bone. Multiple signs can be investigated through these imaging tests. These tests are painless. These test mostly include-
  • Scanning of the bone 
  • Computerised tomography (CT)
  • X-ray
  • Magnetic resonance imaging (MRI)
  • Positron emission tomography (PET)
  1. Biopsy- This is a procedure in which a sample of a tumour cell is taken out for lab testing. By conducting biopsy the nature of the tumour cells can be confirmed. As in whether they are malignant or benign and accordingly the treatment is decided. Biopsy can be of two types. Needle Biopsy and surgical biopsy. 
  • Needle Biopsy- In this procedure a thin, hollow needle is inserted in the body to extract a few of the tumour cells. 
  • Surgical Biopsy- Surgical biopsy can be of two  types. One is excisional biopsy in which the whole of the tumour is removed and then tested. Another is Incisional biopsy in which a portion from the tumour is removed and tested. 

The findings of  biopsy results are very valuable information for the determination of the suitable course of treatment. 

  1. The cancer cells are tested for the genetic mutation that results in Ewing sarcoma. As explained earlier in case of Ewing Sarcoma translocation of chromosomes takes place which results in formation of a new gene called EWS-FLI1. Detection of this gene in the cells helps in conformity of the disease along with the aggressiveness of the same. 

Symptoms- As we know that Ewing Sarcoma is found more in children and teenagers. The signs and symptoms that are common among the patients are unexplained tiredness, prolonged bone pains, pain, swelling or tenderness around the affected area. Fever without any known reason and unexplained weight loss without any effort. Any of such symptoms should not be ignored and immediately checked with the doctor to rule out any adverse results. 

Treatment- The treatment of Ewing sarcoma usually starts with chemotherapy followed by surgically eliminating the tumour or vice- versa. In the treatment of chemotherapy powerful drugs are used to kill the cancer cells. Another treatment that is used to treat Ewing Sarcoma is radiotherapy. In radiotherapy the cancer cells are killed with the help of radiation. Radiotherapy is also used after surgery to kill the remaining cancer cells. Or it can be used in cases where surgery cannot be performed. 

When talking about the treatment of cancer, or any disease for that matter, we cannot rule out India as the land of medicinal knowledge and resources. Known for their medical tourism, expertise, and relatively high success rates, Indian hospitals, especially those in Mumbai and Bangalore are the best when it comes to rightful diagnosis and treatments. Furthermore, one such hospital is considered as the best cancer hospital in Bangalore because it has helped many cancer patients to recover from this disease. 

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