It usually appears as a lump or swelling in the hands, feet, pelvis, or chest. Ewing's sarcoma most often spreads to the lungs and other bones.
Ewing's sarcoma is a rare but aggressive and malignant tumor that affects the bones or soft tissues, most often affecting the bones of the legs and pelvis. It most often occurs in children, adolescents, and young adults.
This sarcoma represents a group of rare malignant tumors of the bone (bone cancer) and soft tissues, including muscles, tendons and ligaments. Clinically, it presents with pain in the affected bone, local swelling and lumps that may be warm to the touch.
The tumors often develop during puberty, a period of rapid bone growth. They most commonly affect children aged 10–20 years, with diagnosis usually made around the age of 15.
However, the disease can also appear in younger children, as well as in adults in their late 20s and early 30s, the Telegraph reports.
Types of Ewing's sarcoma
There are three types of Ewing's sarcoma:
• Ewing's sarcoma of the bone – the most common form, directly affects the bone.
• Ewing's extraosseous tumor (EOE) – develops in the soft tissues surrounding bones, including muscles, tendons, and ligaments.
• Primitive Peripheral Neuroectodermal Tumor (PPNET) – can form in bone or soft tissue and is related to the same malignant cell line as Ewing's sarcoma.
What parts of the body does Ewing's sarcoma affect?
Ewing's sarcoma can appear in different areas of the body, most commonly in:
• pelvis
• ribs
• shoulder
• thigh
• in the calf of the leg
Although it can affect any bone, it usually starts in the bones of the legs and pelvis. Less commonly, it starts in the soft tissues of the chest, abdomen, arms, or other areas.
Symptoms of Ewing's sarcoma
Signs and symptoms usually begin in or around the affected bone. It may appear as a lump or swelling in the hands, feet, pelvis, or chest.
Other symptoms may include:
• Bone pain that is intermittent and often worse at night
• Swelling of the tissues around the affected bone, accompanied by pain when touched
• Palpable lumps near the surface of the skin, which may be warm and tender
• Persistent fever that doesn't go away
• Spontaneous fractures (bone breaks) without prior trauma
• Fatigue and unexplained weight loss, especially if the tumor metastasizes
Causes of Ewing's sarcoma
Ewing's sarcoma is caused by a somatic genetic mutation that occurs after birth. It most often involves the splicing of genes EWSR1 and FLI1, creating a hybrid gene that promotes uncontrolled cell division and malignant tumor formation.
The initial cause of the mutation is unknown. Studies have not found a link to long-term exposure to carcinogens, as the disease mainly affects children and young adults, who are usually not exposed to cancer-causing substances for a long time.
The two risk factors for Ewing's sarcoma
According to the American Cancer Society, the main risk factors are:
• Age – most common in children and adolescents 10–20 years old
• gender – occurs slightly more often in men than in women
Complications of Ewing's sarcoma
Complications may include:
• Relapse of the disease after treatment
• metastases, especially in the lungs and other bones
• Other malignant tumors independent
Late effects of treatment may appear months or years later and may affect:
• Organs and tissues
• Growth and development
• Mental health and mood
• Cognitive functions (thinking, learning, memory)
• Reproductive ability
How is Ewing's sarcoma diagnosed?
Diagnosis involves a physical examination and a detailed evaluation of symptoms. The most common tests are:
• Imaging tests: CT, MRI, PET and bone scanner
• Biopsy: microscopic analysis of the affected tissue (with needle or bone marrow biopsy)
• Blood tests: complete blood count, LDH and other tests that assess the spread of the disease
How is Ewing's sarcoma treated?
Treatment usually includes a combination of:
• Chemotherapy – often as initial treatment to shrink the tumor
• surgery – for tumor removal after chemotherapy
• Radiotherapy – as additional or alternative therapy
• Autologous stem cell transplant, in selected cases when the tumor has not affected the marrow
Some patients may benefit from participating in clinical studies, which test new treatments or more effective combinations.
When should you seek medical help?
You should consult a doctor if you experience:
• Severe side effects from treatment
• Unexplained pain that does not go away with analgesics
• Worsening of existing symptoms or appearance of new symptoms
Survival rate in Ewing's sarcoma
According to the American Cancer Society:
• Localized tumor: 81%
• Regional tumor (spread to nearby tissues): 77%
• Advanced/metastatic tumor: 41%
These statistics are general estimates and do not predict individual patient progress.
How to support your child
Diagnosing cancer is difficult at any age, but especially in Ewing's sarcoma, which most often affects those aged 10–20. Treatment can cause anxiety in the patient and fear or uncertainty in parents.
The needs of a child with this diagnosis change during the treatment and recovery process. Puberty can make this period even more difficult, affecting self-confidence and emotions.
Parents and caregivers need to be patient and understanding - both with their child and with themselves. If they feel overwhelmed, the oncology team can offer support, advice and personalized information. /Telegraph/
