Ewing’s Sarcoma Flashcards
What is the epidemiology of Ewing’s Sarcoma?
Ewing’s Sarcoma is the 2nd most common bone tumor in children.
What percentage of bone tumors does Ewing’s Sarcoma account for?
Ewing’s Sarcoma accounts for approximately 16% of bone tumors.
What percentage of childhood cancers is Ewing’s Sarcoma?
Ewing’s Sarcoma accounts for approximately 3% of childhood cancers.
What is the typical age range for Ewing’s Sarcoma occurrence?
Ewing’s Sarcoma occurs in most patients between 10 - 20 years old.
What is the age range in which Ewing’s Sarcoma can affect individuals?
Ewing’s Sarcoma can affect anyone from the age of 5 months to 60 years.
Is Ewing’s Sarcoma common in children younger than 5?
Occurrence in children younger than 5 is rare.
In which racial group is Ewing’s Sarcoma more common?
Ewing’s Sarcoma is more common in White Caucasians.
Is Ewing’s Sarcoma common in Asian or African children?
It is unusual for this tumor to affect Asian or African children.
Which gender is more commonly affected by Ewing’s Sarcoma?
Ewing’s Sarcoma is more common in males than females.
What is Ewing’s Sarcoma composed of?
Ewing sarcoma is composed of a population of small, blue, round cells with a high nuclear to cytoplasmic ratio.
What is the exact cause of Ewing’s Sarcoma?
No exact cause has been identified, but there is a strong association with genetic alterations.
What is a genetic epidemiological risk factor for Ewing’s sarcoma?
In 40%-70% of cases, patients with Ewing sarcoma have been found to have a loss of heterozygosity in a chromosomal event.
What genetic alteration is commonly associated with Ewing’s Sarcoma?
The next most common alteration affects a tumor protein, p53, which arises from the TP53 gene.
What are some risk factors for Ewing’s Sarcoma?
Risk factors include gender, race, and height.
What is the most common clinical presentation of Ewing’s Sarcoma?
Pain
Delays in diagnosis can occur from an absence of pain.
What are other clinical presentations of Ewing’s Sarcoma?
Swelling, Fever, Weight loss, Fatigue, Limitation of movement.
Why might patients delay seeking medical attention for Ewing’s Sarcoma?
Patients may prolong seeking medical attention until the lesion changes in size.
Where is Ewing’s Sarcoma most frequently seen?
In the appendicular skeleton, with about 25% of tumors developing in the pelvis and about 17% affecting the femur.
In which part of the bone does Ewing’s Sarcoma most commonly occur?
Most commonly seen in the diaphysis.
What are the main treatment modalities for Ewing’s Sarcoma?
Surgery, chemotherapy, and radiation therapy.
How is chemotherapy used in the treatment of Ewing’s Sarcoma?
Often used FOLLOWING surgery to improve overall survival. Common agents include vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide.
What is the role of radiation therapy in Ewing’s Sarcoma treatment?
Typically given postoperatively, especially in cases with positive surgical margins.
What is the goal of treatment for patients with Ewing Sarcoma?
Eradication of the entire tumor and preservation of as much function as possible.
What is the recommended radiation dose for curative treatment of Ewing Sarcoma?
55-60Gy total at 1.8 Gy/fraction.
What is the recommended timing for starting EBRT in postoperative cases in Ewing Sarcoma?
It is recommended to start EBRT within 60 days of surgery for optimal response.
What is the significance of shrinking the tumor before surgery?
Increases the likelihood of achieving clear margins with complete resection.
What is the typical radiation dose for preoperative treatment of Ewing sarcoma?
Lower doses ranging from 36-45 Gy.
What is the typical radiation dose for postoperative treatment of Ewing sarcoma?
Lower doses ranging from 45 to 55.8 Gy depending on the level of resection.
What technique is recommended for radiation treatment planning of Ewing sarcoma?
Shrinking field technique with a 2 cm margin around the primary lesion.
What should be spared during radiation therapy to reduce late effects?
Spare the non-involved epiphysis and a lateral strip of tissue to prevent lymphedema.
What is the most important prognostic factor for Ewing sarcoma?
The extent of disease at the time of diagnosis.
How does metastatic disease at diagnosis affect prognosis?
Patients with metastatic disease usually have a poor outcome.
How does bone or bone marrow involvement affect prognosis?
Patients with bone or bone marrow involvement did not do as well as those with limited pulmonary involvement.
What is the prognosis for patients with soft tissue extension?
Extension into soft tissue is associated with a less favorable prognosis than limited or no soft tissue involvement.
What impact does tumor size have on prognosis?
Large tumors and those arising in the pelvis can negatively affect prognosis.
What is the recurrence rate for Ewing sarcoma?
The recurrence rate is 30% to 40%, which includes local or distant disease.
