Treatment of Ewings sarcoma abroad
Ewing’s sarcoma or endothelial myeloma is a malignant tumour that develops from the stromal cells of the bone marrow. Primary target of the tumour are bones. Illness has a negative prognosis as tumour grows fast and has early metastases.
In general, Ewing’s sarcoma is not common. Though, sarcoma is second on frequency malignant oncopathology among all the paediatric tumours.
Causes of Ewing's sarcoma
Ewing’s sarcoma develops during adolescence and can rarely appear in youth or childhood. This tumour is rare among the elderly people.
Basic risk factors are:
- Race ( Ewing’s sarcoma is rarely detected among people of African origin)
- Male sex
- Certain gene and chromosomal mutations
A background of hereditary is absent and environmental conditions pose no threat to a person in this regard.
Symptoms of Ewing's sarcoma
Clinical signs of the tumour depend on its location. Most often Ewing’s sarcoma is detected in the bones of:
- Lower limbs and pelvis in 70% of cases
- Upper extremities in 17% of cases
- Other places (ribs, shoulder blades, collar bone, skull) in 13%
Pain is mild at the beginning of the illness, but it worsens as the disease grows. Pain increases at night. Limb or body part fixation doesn’t relieve the pain. Pain can disappear by itself for several days or weeks, but then it comes back again.
Soft-tissue component. You can observe swelling and redness in the area affected by Ewing’S sarcoma. Subcutaneous veins are expanded. The function of the corresponding limb or body part might be hindered. Swelling can be the first sign of the illness and a reason to get a doctor’s appointment.
Intoxiaction syndrome is indicated by fever, sometimes up to 39-40 degrees celsius, weakness, headaches, weight loss.
Pathological bone fractures are observed among 5% of patients. The likelihood of fractures also depends on the location of the sarcoma. When the tumour is located in the proximal femur, the probability of its fracture is 70%.
Other symptoms depend on the location of oncological process:
- Patient might be disturbed by claudication when tumor grows in the lower limbs
- Pain, numbness of certain body parts says about tumor in the spinal cord, depending on the degree of damage. Movement disorders and violated functions of the pelvic organs can also appear
- Ewing’s sarcoma in the chest bones can cause shortness of breath, cough, and hemoptysis due to the possible accumulation of fluid in the pleural cavity
Diagnostics of Ewing's sarcoma
Following visualization methods will help to identify the pathological changes of the bones, tumor and its metastases:
- Computed tomography
- Magnetic resonance imaging
- Positron-emission tomography
To confirm a diagnosis, they need biopsy with the further testing of the tumor tissues:
- Using a PCR (polymerase chain reaction)
Biopsy can be excisional or incisional. Excisional biopsy is rarely applied as it requires the removal of the whole tumor. It’s possible at the beginning stage of Ewing’s sarcoma, when malformation has small size and is located in the convenient place for surgical resection.
Incisional biopsy means taking tumor fragment for analysis. The procedure is held by one of the following ways:
- Closed – with the help of needle
- Opened – through the incision (surgical intervention)
In some cases, bone marrow biopsy is needed, as Ewing’s sarcoma often gives metastases over there.
Other ways that can be used for diagnostics are:
- Bone scan (for detecting bone metastases)
Treatment of Ewing's sarcoma abroad
Basic methods for Ewing’s sarcoma treatment are:
- Radiation therapy
- Surgical intervention
Surgeries can be different depending on the location of the tumour. If tumour is extensive, then surgery is not performed. Instead, doctors prescribe only chemotherapy and radiation therapy.
They remove a bone and switch it with a prosthesis when sarcoma is located on the limbs. Kids need to have this prosthesis changed every several years to match their growth. There are high-tech prosthesis that can change its size without any surgery. A limb is rarely amputated if sarcoma is in the bones.
If the tumour is located in the chest, surgeons remove the tumour and closely located ribs. Often, Ewing’s sarcoma spreads into the lungs and thus, the surgery expands to those areas as well.
Tumours of the pelvic cavity require the removal of bone fragments. Regardless of tumour location, they remove the soft-tissue together with the bone. Meanwhile, the surgeon tries to preserve nerves and tendon as much as possible to ensure the mobility of the operated body part.
Ewing's sarcoma - Prognosis
Ewing’s sarcoma has a negative prognosis. It depends, first of all, on whether the tumour has already metastasised or not.
Five-year survival of the patients is:
- 70% if no metastases were detected
- From 15 to 30% if there are metastases
If there are metastases, a number of other factors have to be considered to define the prognosis. It will be more favourable, if metastases is detected only in the lungs, but not in the other organs.
Other positive prognostic factors for Ewing’s sarcoma are:
- Age up to 10 years
- LDH in the blood in the norm limits
- Major tumour is on the arm or leg
- Tumour responds to chemotherapy
The bigger the tumour, the worse is prognosis. Tumour location in the pelvic bones or chest bones is considered to be among the less favourable prognostic factors.