Ewing sarcoma is a malignant neoplasm that develops from bone tissue. It most often occurs in adolescence. It affects short (feet and hands) and long (extremities) tubular bones. It is one of the most aggressive metastasis-prone tumors.
Ewing sarcoma is a form of bone cancer that is characterized by rapid growth and early formation of metastases. It usually develops in children, adolescents, and young adults.
Ewing sarcoma is the second most common form of bone cancer that occurs in childhood, accounting for 10 to 15% of the total number of bone cancer cases. It is rare in adults because the developing bone tissue is a favorable environment for Ewing sarcoma onset.
In some cases, the primary neoplasm in this disease occurs in soft tissues, and not in bones. This form of bone cancer is called extraosseous Ewing sarcoma. Also, there are aggressive malignant tumors that are similar in nature and structure and belong to the Ewing sarcoma group of bone cancer. This group, in addition to extraosseous and bone sarcoma, includes PNET (peripheral primitive neuroectodermal tumors). Extraosseous forms and PNET together account for about 15% of the total number of cases of Ewing sarcoma.
The first and earliest clinical sign is the specific pain in the affected area, mostly bone pain. At first, there’s a minor pain that disappears spontaneously, and then appears again. Unlike pain that is common for other types of cancer, pain in Ewing sarcoma does not diminish at rest or at night. Moreover, Ewing's sarcoma is characterized by pain that is worse at night.
As the pain progresses, it becomes more intense, deprives patients of sleep, and limits daily activities. There are symptoms like a local increase in the blood flow and enlargement of veins of the affected area.
Local clinical signs are combined with increasing symptoms of general tumor intoxication. Patients complain of general weakness or fatigue and loss of appetite. There is a decrease in body mass, up to exhaustion. Regional enlargement of one or several lymph nodes also belongs to the symptoms of Ewing sarcoma.
Some symptoms depend on the location of this type of bone cancer. So, when Ewing sarcoma occurs in the lower extremities, the impaired functioning of the bones of the lower extremities occurs. With damage to the vertebrae, development of myelopathy (compression of the spinal cord) with dysfunction of the pelvic organs and the impaired functioning of the lower extremities occurs. With neoplasms in the chest bones, respiratory failure, the presence of excess fluid in the lungs, and coughing up blood may occur.
The tumor grows rapidly (usually several months after the onset of the first symptoms) and becomes so large that it can be easily felt. At the later stages, a pathological fracture often occurs in the area of bone cancer.
The first-line clinical examination for Ewing sarcoma is an X-ray of the area of concern. When clinical signs of Ewing's sarcoma are detected on X-ray, the patient is referred to the oncology department, where a comprehensive examination is carried out to assess type, and the process of development of bone cancer. In the course of such an examination, CT scan and MRI of bones and soft tissues affected by bone cancer are performed. These clinical studies make it possible to determine the size of the Ewing sarcoma, its spread to the bone marrow, and its connection with the neurovascular bundle and surrounding tissues.
To detect metastases in the lungs, computed tomography and radiography of the lungs are used. To detect metastases in the bones, bone marrow, and internal organs, positron emission tomography, and ultrasound scan are performed. Also, some additional medical studies are indicated to determine the type of bone cancer.
A biopsy is also performed. The material is harvested from a site of bone near the bone marrow, or, if this is not possible, from the soft tissue component of the tumor. Since Ewing sarcoma is characterized by local and distant damage to the bone marrow, a bone marrow aspiration is performed, during which bone marrow tissue is harvested. To assess the nature of the process, immunohistochemical analysis and molecular genetic studies can also be carried out.
The treatment options for Ewing sarcoma include surgery, preoperative and postoperative chemotherapy, radiation therapy, and bone marrow transplantation.
However, since this tumor belongs to the category of highly aggressive neoplasms, and is characterized by early metastasizing, treatments should be systemic, even in cases of absence of the metastases. The fact is that there is a very high probability of the early appearance of micrometastases, which cannot be detected at the time of diagnosis making.
The choice of the method of surgery depends on the location of bone cancer and the prognosis of the functioning of the affected part of the body after surgery.
If bone cancer is located in the chest, it is removed along with several ribs, which are replaced with synthetic material. Cancer cells in the lungs are removed during the surgical intervention as well. After the operation, such patients undergo radiation therapy. In patients with Ewing sarcoma with damage to the vascular system, radiation therapy could be used as an alternative to surgery.
In many cases, the affected bone or soft tissue can be completely removed by surgery without affecting organ function. In other cases, the preservation of organ function after the complete removal of the tumor tissue is impossible.
If surgical treatment for bone cancer is carried out at the advanced stages, poor wound healing and infectious complications can develop. This is due to the previously performed chemotherapy and radiation therapy. After the surgical treatment, many patients with Ewing sarcoma require rehabilitation treatments.
Ewing sarcoma is highly sensitive to radiation and most patients can be treated with a combination of chemotherapy and radiation therapy.
However, radiation therapy can have specific side effects.
Bone growth disorder is a serious complication of radiation therapy. Irradiation of the pelvic area may damage the bladder and colon. When applied to the spinal cord and brain, radiation therapy may cause the worsening of the health condition that manifests itself with headaches. Moreover, the younger the patient is, the more pronounced the side effects and complications are.
In 20% of cases, high doses of radiation lead to the development of a secondary malignant tumor. So, radiation therapy in Ewing sarcoma treatment is usually reserved for very serious cases.
Chemotherapy in patients with Ewing sarcoma is carried out with the help of anti-cancer drugs that are usually administered intravenously.
This method of treatment is indicated for all patients, regardless of the degree of bone cancer progression. This is because patients with a localized process may have micrometastases that are detected only by microscopic examination. Therefore, refusal of chemotherapy in patients with Ewing sarcoma will eventually lead to the growth of these micrometastases.
Chemotherapy can cause various side effects, including nausea, vomiting, loss of appetite, increased susceptibility to infections, menstrual irregularities, and damage to the heart muscle.
Recently, patients with a poor prognosis, in particular those with bone cancer metastases, with a survival rate of less than 10%, have been receiving more intensive treatments consisting of high-dose chemotherapy and radiotherapy, and transplantation of bone marrow. This approach allows curing more than 30% of patients with metastatic processes.
The prognosis for Ewing sarcoma depends on many factors. If metastases are found, the prognosis worsens significantly. However, the complex therapy can successfully treat patients with advanced forms of bone cancer in 30% cases.
After recovery, all patients should undergo regular check-ups for early detection of relapses and control of side effects. It should be borne in mind that some side effects can occur not only during treatment, but also long after its completion. However, many patients treated for Ewing sarcoma can live full lives.
Here’s the list of hospitals specialized in the diagnostics and treatment of Ewing sarcoma:
If you consider any of the listed hospitals for treatment, leave the request on the Booking Health website.
The cost of treatment depends on the country patients undergo therapy for bone cancer in, on the pricing policy of the hospitals, and the medical procedures in each particular case.
Generally, the cost of treatment of Ewing sarcoma with chemotherapy and radiotherapy in Germany starts at 32,181 EUR, and in Turkey it starts at 25,574 EUR.
The cost of treatment with surgical intervention in Germany starts at 16,533 EUR, and in Turkey – at 11,899 EUR.
The price for diagnostics alone in Germany starts at 5,714 EUR, and in Turkey – at 3,813 EUR.
There’s more information about treatment prices on the Booking Health website.
Unfortunately, hospitals in some countries are not able to provide proper cancer care, especially when it comes to such rare and complex health conditions as Ewing sarcoma. Besides, the treatment methods and technologies usually lag behind the foreign ones.
Recently, more and more people are thinking about traveling to other countries to treat various types of cancer and receive qualified health care, diagnostics, as well as having a second opinion. Often, it is the right thing to confirm the diagnosis and treatment correctness in foreign hospitals.
So, treatment abroad is clearly a good option for many cancer patients, but how can one go for treatment abroad? Well, instead of going through countless sources on the topic, you can contact the medical tourism agency Booking Health.
Booking Health will help you select a specialized medical facility for your diagnosis among the best hospitals in the world, will take upon all the organizational issues, and will assist you throughout your journey for treatment.
For what it's worth, it is much more comforting not to do it alone. To find out how to start your treatment, leave the request on the Booking Health website.
Authors: Dr. Nadezhda Ivanisova, Dr. Farrukh Ahmed
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