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EWING SARCOMA

Ewing Sarcoma is a type of highly aggressive cancer that grows in any bone in the body. However, it most commonly affects the lower extremities. It includes the legs and the pelvic region. It affects mostly young children or teenagers and is more common in male than female. Experts also refer to it as the peripheral primitive neuroectodermal tumor.

Ewing Sarcoma affects one person in every million. It seems to be more prominent among the white race. The little percentage of Ewing Sarcoma that is present in African is due to a faulty gene. Experts suggest that polymorphism of the EGR2 gene in Africans can cause the disease. It affects the male population more than females. Moreover, 50% of people with the disease are adolescents.

Types of Ewing Sarcoma

The type of Ewing Sarcoma depends on the body part that it affects. It most commonly starts in the pelvis. The tumor may progress towards the femur next. Irrespective of the body part that it affects, it can eventually affect other bones. Bone marrow is one of the most sensitive parts of the body that it affects in its progressive stage. Some of the types of Ewing Sarcoma are as follows;

Bone Tumors

87% of the Ewing’s Sarcoma patients are affected in the bones. It usually involves the femur, ribs, pelvis, and shoulder blades.

Extraosseous Sarcoma

This starts in the soft tissues around the bones. It involves the cartilage and the muscles. Most commonly present in the soft tissues of the upper and lower extremities.

Peripheral primitive neuroectodermal tumor (pPNET)

This type of Ewing Sarcoma affects the nerves. Because of the involvement of nerves, it can affect any body part.

Askin Tumor

It is a subtype of pPNET tumor. It is common in children and yound adults  and it most commonly affects the chest wall.

Symptoms of Ewing Sarcoma

  • Patients who suffer from Ewing Sarcoma usually portray local symptoms. It includes
  • A palpable mass or swelling
  • Bone pain
  • Fractures due to metastasis
  • Inability to walk properly
  • In patients where the tumor is present in the chest wall, pleural infiltration can occur.
  • Other findings include anemia and leukocytosis. In some instances, the blood reports are similar to osteomyelitis. Because of this, there is a chance of a missed diagnosis by the doctors.

Causes of Ewing Sarcoma

The main causative factor for Ewing Sarcoma is still not clear to experts. However, one thing is certain that there is the involvement of genetic modifications. Most probably, the EWSR1 gene is the reason that causes the disease in children.

Diagnosis

A series of tests help in the diagnosis of the disease. The doctor may stage the disease based on its spread from local origin. The only possibility is after confirmation of the disease.

Physical Examination

At first, the doctor will physically examine the patient for mass or lumps. It includes an examination of any probable areas that are painful.

X-ray

X-ray is a diagnostic imaging test that helps the doctor to take a look at dense tissues inside the body. The doctor will specifically examine areas where the doctor suspects a tumor is

Bone Scan

This helps in the identification of cancerous cells in the bones. The radiologist injects a small dye in the veins. The dye gathers in areas where tumors are present. The bone scanner detects these areas on the screen.

Other Diagnostic Imaging Tests

Other advanced tests that the doctor may perform include MRI or CT scans. MRI scans are better than X-ray or CT scans. It is the reason that some doctors skip X-ray and directly request for an MRI.

PET scan is also a diagnostic imaging test. It helps in the diagnosis of tumors with the help of radioactive substances. Cancerous cells attract this radioactive substance and light up on scans.

Biopsy

The doctor performs a biopsy after confirmation of the tumor. This will help to the stage the Ewing Sarcoma.

Staging

For treatment, the doctors utilize a simpler form of the staging of the disease. According to this simpler form of staging, there are two stages of Ewing Sarcoma.

Localized Ewing Sarcoma

According to this stage, the tumor has not spread from the area of origin. This type of Ewing Sarcoma is relatively easier to treat.

Metastatic Ewing Sarcoma

Based on this stage, the tumor has spread from its area of origin. Some of the parts that it can invade include lungs, bone marrow, or the lymph nodes.

Another staging system exists other than this simpler one. AJCC is responsible for the development of this staging system. This classification system depends on four factors;

  • T: It refers to the primary tumor and its location of origin.
  • N: It refers to the extent that it has spread to. It usually involves the lymph nodes.
  • M: This is an indication that cancer has metastasized to the other parts of the body.
  • G: This refers to the grade of the tumor according to the results of the biopsy samples.

Treatment

The treatment strategies depend on the factors of the AJCC classification system. The selection of treatment options is possible with a single form of therapy. Doctors can offer a combination of treatment options. However, this is only done when the disease has metastasized.

  • Radiotherapy is the standard choice of treatment for patients with Ewing Sarcoma.
  • Currently, treatment of Ewing Sarcoma is possible with the help of multiple treatment methods. It involves chemotherapy, radiotherapy as well as surgery.
  • If the doctor feels that the tumor is difficult to remove surgically, they may prescribe radiotherapy. This preoperative radiotherapy helps in reducing the size of the tumor so the tumor can be removed.
  • Preoperative chemotherapy also helps in shrinking the size of the tumor. The main aim of this chemotherapy is to reduce the micrometastases. It is also helpful in the reduction of the volume of the tumor. Currently, the administration of VDC and IE are preferred for short-term chemotherapy.

REFERENCES

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