extraosseous Ewing sarcoma

What is Extraosseous Ewing Sarcoma?

Extraosseous Ewing sarcoma (EES) is a rare and aggressive type of cancer that originates from soft tissues, rather than bones. It is a member of the Ewing sarcoma family of tumors (ESFT), which also includes skeletal Ewing sarcoma.

Characteristics of Extraosseous Ewing Sarcoma

• Rare occurrence: EES accounts for only 30% of all ES cases.
• Soft tissue origin: EES originates from soft tissues, such as muscles, fat, and connective tissue.
• Aggressive nature: EES is a highly malignant tumor with an aggressive growth pattern.
• Poor prognosis: EES has a poor prognosis, especially in metastatic forms, with high mortality rates.

Symptoms of Extraosseous Ewing Sarcoma

• Atypical symptoms: Presenting symptoms are often atypical and may include:
  • Haematuria (blood in the urine)
  • Abdominal pain
  • A palpable mass

Demographics Affected by Extraosseous Ewing Sarcoma

• Young people affected: EES mainly affects young people, with a peak incidence in children and young adults.
• Chest, pelvis, thigh, foot, or spine commonly affected: EES usually occurs in the chest, pelvis, thigh, foot, or spine.

References:

• [1] Extraosseous Ewing sarcoma (EES) is an uncommon member of the Ewing sarcoma family of tumors (ESFT), characterized by its aggressive nature and poor prognosis. ([7])
• [2] EES accounts for only 30% of all ES cases. ([9])
• [3] EES originates from soft tissues, such as muscles, fat, and connective tissue. ([3])
• [4] EES has a poor prognosis, especially in metastatic forms, with high mortality rates. ([8])

Common Signs and Symptoms of Extraosseous Ewing Sarcoma

Extraosseous Ewing sarcoma (EOE) is a rare type of cancer that affects the soft tissues surrounding bones. The symptoms of EOE can vary from person to person, but here are some common signs and symptoms:

• Pain: Pain in the affected area is one of the most common symptoms of EOE. This pain can be dull or sharp and may worsen over time.
• Swelling: Swelling and tenderness around the site of the tumor are also common symptoms of EOE.
• Fever: Some people with EOE may experience fever, which can be a sign that the cancer is spreading.
• Weight loss: Unintentional weight loss can occur in some cases of EOE due to the body's increased metabolic rate as it tries to fight the cancer.

Other Possible Symptoms

In addition to these common symptoms, other possible signs and symptoms of EOE include:

• Haematuria: In some cases, people with EOE may experience bleeding from the urinary tract (haematuria).
• Abdominal pain: Pain in the abdominal area can occur if the tumor is located near the digestive organs.
• Palpable mass: A palpable mass or lump may be felt in the affected area.

Important Note

It's essential to seek medical attention immediately if you experience any of these symptoms, as early diagnosis and treatment are crucial for effective management of EOE.

Diagnostic Tests for Extraosseous Ewing Sarcoma

Extraosseous Ewing sarcoma, a rare and aggressive type of cancer, requires prompt and accurate diagnosis to determine the extent of cancer spread. The following diagnostic tests are commonly used to diagnose extraosseous Ewing sarcoma:

• Imaging tests: These tests create images of the body's internal structures, helping doctors identify the location and size of the tumor. Tests may include:
  • X-ray: Uses electromagnetic energy beams to produce images of internal tissues, bones, and organs on film [14].
  • MRI (Magnetic Resonance Imaging): Shows details of the tissue around the tumor, as well as the inside of the bone with the tumor [7][8].
  • CT (Computed Tomography) scan: Creates detailed cross-sectional images of the body, helping doctors identify the extent of cancer spread [7][15].
• Genomics testing: Some newer lab tests can look at all of the genes inside cancer cells at the same time, which can sometimes help guide treatment of the cancer [2].
• Blood tests: Blood tests may be used to get an idea of overall health and show how well the bone marrow is functioning. Tests might include:
  • Complete blood count (CBC): Shows abnormalities in the blood that might suggest the spread of cancer to the bone marrow [4

Treatment Options for Extraosseous Ewing Sarcoma

Extraosseous Ewing sarcoma, a rare and aggressive form of cancer, requires effective treatment to manage its progression. While surgery and radiation therapy are often used in conjunction with chemotherapy, the specific drug treatments employed can vary depending on the patient's condition.

Chemotherapy Regimens

For extraosseous Ewing sarcoma, chemotherapy is typically administered as part of a multimodal treatment approach. The most commonly used chemotherapeutic agents include:

• Vincristine: A vinca alkaloid that interferes with microtubule function, ultimately leading to cell death [9].
• Dactinomycin: An anthracycline antibiotic that intercalates DNA strands, preventing transcription and replication [9].
• Cyclophosphamide: An alkylating agent that cross-links DNA, causing damage and apoptosis [9].
• Doxorubicin: Another anthracycline antibiotic that intercalates DNA, leading to cell death [9].

Targeted Therapy

In some cases, targeted therapy may be employed as part of the treatment regimen. Bevacizumab, a monoclonal antibody targeting vascular endothelial growth factor (VEGF), has been investigated in clinical trials for its potential efficacy against Ewing sarcoma [5]. Additionally, pazopanib, a tyrosine kinase inhibitor, has shown promise in treating metastatic extraosseous Ewing sarcoma [12].

Current Research and Future Directions

Recent clinical trials have highlighted the importance of contemporary drug therapy in managing Ewing sarcoma. Novel therapies, such as CAR T-cell therapy, are being actively investigated for their potential to improve treatment outcomes [5]. The current review emphasizes the need for continued research into effective treatments for this rare and aggressive form of cancer.

References

• [1] Multidisciplinary therapy, such as surgery, radiation therapy, and chemotherapy, generally improves the symptoms and outcomes of Ewing sarcoma; however, metastatic Ewing sarcoma is usually difficult to control.
• [3] Ewing sarcoma most often forms in the bones of the legs, arms, feet, hands, chest, pelvis, spine, or skull.Less often, it forms in the soft tissue of the trunk, arms, legs, head, neck, retroperitoneum (area in the back of the abdomen behind the tissue that lines the abdominal wall and covers most of ...
• [9] Various medications are used in chemotherapy treatment for Ewing's sarcoma. The most common drugs are: Vincristine; Dactinomycin; Cyclophosphamide; Doxorubicin ...
• [12] We herein report a case of heavily pre-treated metastatic extraosseous Ewing sarcoma in which pazopanib treatment achieved a significant improvement. A 17-year-old girl was referred to our hospital due to metastatic extraosseous Ewing sarcoma.
• [14] Ewing sarcoma treatment most often includes chemotherapy and surgery. Which treatment you have first will depend on your situation. Other treatment options might include radiation therapy and targeted therapy. Chemotherapy treats cancer with strong medicines.