Ewing’s sarcoma is characterized by the development of a malignant tumor in the bones and soft tissues. This tumor has the characteristic of being with high metastatic potential. Either the spread of tumor cells throughout the body is often identified in this pathology.

It is a rare disease more generally affecting children. Its incidence amounts to 1/312 children under 500 years of age.

The age group most affected by the development of this tumor form is between 5 and 30 years old, with an even greater incidence between 12 and 18 years old. (3)

The associated clinical manifestations are pain and swelling at the location of the tumor.

The locations of tumor cells characteristic of Ewing’s sarcoma are multiple: legs, arms, feet, hands, chest, pelvis, skull, spine, etc.

This Ewing sarcoma is also called: primary peripheral neuroectodermal tumor. (1)

Medical examinations allow the possible diagnosis of the disease and determine its stage of advancement. The most commonly associated examination is a biopsy.

Specific factors and conditions can impact the prognosis of the disease in an affected subject. (1)

These factors include in particular the spread of tumor cells to the lungs only, the prognosis of which is more favorable, or the development of metastatic forms to other parts of the body. In the latter case, the prognosis is poorer.

In addition, the size of the tumor and the age of the affected individual have a fundamental role in the vital prognosis. Indeed, in the case where the size of the tumor rises to more than 8 cm, the prognosis is more worrying. As for age, the earlier the diagnosis of the pathology is made, the better the prognosis for the patient. (4)

Ewing’s sarcoma is one of the three main types of primary bone cancer along with chondrosarcoma and osteosarcoma. (2)

The symptoms most commonly associated with Ewing’s sarcoma are visible pain and swelling in the affected bones and soft tissues.

 The following clinical manifestations may be originating in the development of such a sarcoma: (1)

• pain and / or swelling in the arms, legs, chest, back or pelvis;
• the presence of “bumps” on these same parts of the body;
• the presence of fever for no specific reason;
• bone fractures for no underlying reason.

The associated symptoms nevertheless depend on the location of the tumor as well as its importance in terms of development.

The pain experienced by the patient with this pathology usually intensifies over time.

 Other, less common symptoms may also be visible, such as: (2)

• a high and persistent fever;
• muscle stiffness;
• significant weight loss.

However, a patient with Ewing’s sarcoma may not have any symptoms. In this sense, the tumor can then grow without any particular clinical manifestation and thus affect the bone or the soft tissue without being visible. The risk of fracture is all the more important in the latter case. (2)

As Ewing’s sarcoma is a form of cancer, little is known about the exact origins of its development.

A hypothesis was nevertheless put forward relating to the cause of its development. Indeed, Ewing’s sarcoma particularly affects children over 5 years old and adolescents. In this sense, the possibility of a link between rapid bone growth in this category of person and the development of Ewing’s sarcoma has been raised.

The period of puberty in children and adolescents makes bones and soft tissues more vulnerable to the development of a tumor.

Research has also shown that a child born with an umbilical hernia is three times more likely to develop Ewing’s sarcoma. (2)

Beyond these hypotheses mentioned above, the origin as to the presence of a genetic translocation has also been put forward. This translocation involves the EWSRI gene (22q12.2). A t (11; 22) (q24; q12) translocation within this gene of interest was found in nearly 90% of tumors. In addition, many genetic variants have been the subject of scientific investigations, involving the ERG, ETV1, FLI1 and NR4A3 genes. (3)

From the point of view where the exact origins of the pathology are, to this day, still poorly known, the risk factors are also.

In addition, according to the results of scientific studies, a child born with an umbilical hernia would be three times more likely to develop a type of cancer.

In addition, at the genetic level, the presence of translocations within the EWSRI gene (22q12.2) or genetic variants in the ERG, ETV1, FLI1 and NR4A3 genes, may be the subject of additional risk factors for developing the disease. .

The diagnosis of Ewing’s sarcoma is based on a differential diagnosis through the presence of characteristic symptoms in the patient.

Following the doctor’s analysis of the painful and swollen areas, an x-ray is usually prescribed. Other medical imaging systems can also be used, such as: Magnetic Reasoning Imaging (MRI) or even scans.

A bone biopsy may also be done to confirm or not the diagnosis. For this, a sample of bone marrow is taken and analyzed under a microscope. This diagnostic techniques can be performed after general or local anesthesia.

The diagnosis of the disease must be carried out as soon as possible so that the management is done quickly and thus the prognosis is better.

 Treatment for Ewing’s sarcoma is similar to general treatment for other cancers: (2)

• surgery is an effective way to treat this kind of sarcoma. However, the surgical intervention depends on the size of the tumor, its location as well as its level of spread. The goal of surgery is to replace the part of bone or soft tissue damaged by the tumor. For this, a metal prosthesis or a bone graft can be used in the replacement of the affected area. In extreme cases, limb amputation is sometimes necessary to prevent cancer relapse;
• chemotherapy, usually used after surgery to shrink the tumor and facilitate healing.
• radiotherapy, is also often used after chemotherapy, before or after surgery to reduce the size of the tumor and avoid the risk of relapse.