What is Ewing's sarcoma and its' symptoms?

What is Ewing's sarcoma and its' symptoms?

Publication date: 18-07-2023

Updated on: 18-07-2023

Topic: Oncology

Estimated reading time: 1 min

Ewing's sarcoma is a primary malignant tumor that predominantly affects bone, although it can sometimes affect other body districts such as, for example, soft parts. It is a neoplasm that most frequently affects pediatric and young adolescent subjects and is the second most common malignant tumor of bone, preceded in first place by osteosarcoma. 

As a very aggressive and complicated tumor to treat, early diagnosis can be an essential factor in intervening as early as possible and limiting, as much as possible, too heavy consequences. Dr. Alessandro Luzzati, head of the Center for Orthopedic Oncology and Reconstructive Surgery of the Spine (C.C.O.O.R.R.) at Ospedale Galeazzi-Sant'Ambrogio, tells us about it.

Ewing's sarcoma: who does it affect and what parts of the body does it affect?

“Ewing's sarcoma is a type of tumor with no obvious early symptoms that can affect all age groups, but, in particular, the 2 to 18 age group, with the average age being around 15 years” , Dr. Luzzati explains. “Unfortunately, to date, no definite causes responsible for the occurrence of sarcoma have been identified, but we do know, in any case, that it affects male subjects more than female subjects.

The most affected areas are the bones, in particular:

  • pelvis;
  • long bones (e.g., femur);
  • spine.

But sarcoma is not only the preserve of hard parts; sometimes it can also affect the peritoneal soft tissues, such as, for example, muscles or areas surrounding bones."

Symptoms and the importance of differential diagnosis of Ewing's Sarcoma

As for the symptomatology, it is not strange if, in the early stages, the pathology can apparently hide in other similar disorders. Often, Ewing's sarcoma can mimic an infection with localized pain, followed by fever, abnormal blood tests, warm and reddened area. 

“Initially and radiologically” , Dr. Luzzati continues, “it may present with features that would likely relate it to osteomyelitis, which is an infection of the bone. It is a dull ache that can last for several weeks, after which, once the first examinations are done, the first alarm bells that begin to go off in the patient's mind". 

Usually the diagnostic procedure involves some instrumental examinations of the skeletal area, which are useful in detecting and localizing the abnormality:

Definitive diagnosis is obtained by biopsy, an in-depth examination that can establish the nature of the pain and its origin. Because it is, as mentioned, a subtle and infectious disease, it is critical that the report be read by a pathologist who has experience in these diagnoses related to bone diseases”.

Treatment of Ewing's sarcoma

Ewing's sarcoma survival is estimated between 50-60% of cases. The main treatment modalities are:

“Chemotherapy represents the obligatory step to which one undergoes at an early stage, precisely for the purpose of reducing the size of the tumor in the affected area. It is the gold standard”, the specialist says.
The traditional type is used, which involves several cycles ranging from 4 to 6 and 8. Each cycle lasts about 1 month. Upon completion, the tumor is treated locally, through removal of the diseased portion of the bone and reconstruction of the excised area.

Surgery is performed only when possible and if the condition of the tumor permits. In some cases, when the price to pay is beyond the realm of possibility, the option of exclusive high-dose radiation therapy may also be considered, which has the advantage of avoiding the possible damage caused by surgery, but also has the disadvantage of not always being as 100 percent effective as surgery.

Sometimes, a course of low-dose radiation therapy is used first, which can help improve the outcome. Ewing's sarcoma is, in fact, a very radiosensitive tumor: therefore, as a first step, chemo is given, followed by radiation therapy (with affordable dosages), until the neoplasm shrinks to the point where surgery can be performed.

When, on the other hand, the clinical picture is inauspicious, we are talking about patients with a poor prognosis or otherwise difficult to operate, and the consequences of surgery are too heavy, high doses of radiation therapy are used to replace surgery while not having the same radicality. 

When the sarcoma attacks districts in complex locations, such as, for example, the sacrum, removal surgery is not recommended at all because it would not only result in heavy lifelong consequences for the patient (e.g., urinary and fecal incontinence, poor ambulation, and, in the case of a male subject, problems with erectile dysfunctionality), but also the uncertainty of tumor remission. 

After recovery

“In case of a cure, the patient for 10 years is monitored stringently especially for the occurrence of any recurrences in other districts (e.g., leukemia, lymphoma, breast cancer)", Luzzati concludes. “These, in fact, are mainly the consequence of the use of massive doses of chemotherapy to treat sarcoma and for which, unfortunately, the body has had to pay the price years later". 

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