Ewing’s Sarcoma

Question 1

What is the epidemiology of Ewing’s Sarcoma?

Answer 1

Ewing’s Sarcoma is the 2nd most common bone tumor in children.

Question 2

What percentage of bone tumors does Ewing’s Sarcoma account for?

Answer 2

Ewing’s Sarcoma accounts for approximately 16% of bone tumors.

Question 3

What percentage of childhood cancers is Ewing’s Sarcoma?

Answer 3

Ewing’s Sarcoma accounts for approximately 3% of childhood cancers.

Question 4

What is the typical age range for Ewing’s Sarcoma occurrence?

Answer 4

Ewing’s Sarcoma occurs in most patients between 10 - 20 years old.

Question 5

What is the age range in which Ewing’s Sarcoma can affect individuals?

Answer 5

Ewing’s Sarcoma can affect anyone from the age of 5 months to 60 years.

Question 6

Is Ewing’s Sarcoma common in children younger than 5?

Answer 6

Occurrence in children younger than 5 is rare.

Question 7

In which racial group is Ewing’s Sarcoma more common?

Answer 7

Ewing’s Sarcoma is more common in White Caucasians.

Question 8

Is Ewing’s Sarcoma common in Asian or African children?

Answer 8

It is unusual for this tumor to affect Asian or African children.

Question 9

Which gender is more commonly affected by Ewing’s Sarcoma?

Answer 9

Ewing’s Sarcoma is more common in males than females.

Question 10

What is Ewing’s Sarcoma composed of?

Answer 10

Ewing sarcoma is composed of a population of small, blue, round cells with a high nuclear to cytoplasmic ratio.

Question 11

What is the exact cause of Ewing’s Sarcoma?

Answer 11

No exact cause has been identified, but there is a strong association with genetic alterations.

Question 12

What is a genetic epidemiological risk factor for Ewing’s sarcoma?

Answer 12

In 40%-70% of cases, patients with Ewing sarcoma have been found to have a loss of heterozygosity in a chromosomal event.

Question 13

What genetic alteration is commonly associated with Ewing’s Sarcoma?

Answer 13

The next most common alteration affects a tumor protein, p53, which arises from the TP53 gene.

Question 14

What are some risk factors for Ewing’s Sarcoma?

Answer 14

Risk factors include gender, race, and height.

Question 15

What is the most common clinical presentation of Ewing’s Sarcoma?

Answer 15

Pain

Delays in diagnosis can occur from an absence of pain.

Question 16

What are other clinical presentations of Ewing’s Sarcoma?

Answer 16

Swelling, Fever, Weight loss, Fatigue, Limitation of movement.

Question 17

Why might patients delay seeking medical attention for Ewing’s Sarcoma?

Answer 17

Patients may prolong seeking medical attention until the lesion changes in size.

Question 18

Where is Ewing’s Sarcoma most frequently seen?

Answer 18

In the appendicular skeleton, with about 25% of tumors developing in the pelvis and about 17% affecting the femur.

Question 19

In which part of the bone does Ewing’s Sarcoma most commonly occur?

Answer 19

Most commonly seen in the diaphysis.

Question 20

What are the main treatment modalities for Ewing’s Sarcoma?

Answer 20

Surgery, chemotherapy, and radiation therapy.

Question 21

How is chemotherapy used in the treatment of Ewing’s Sarcoma?

Answer 21

Often used FOLLOWING surgery to improve overall survival. Common agents include vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide.

Question 22

What is the role of radiation therapy in Ewing’s Sarcoma treatment?

Answer 22

Typically given postoperatively, especially in cases with positive surgical margins.

Question 23

What is the goal of treatment for patients with Ewing Sarcoma?

Answer 23

Eradication of the entire tumor and preservation of as much function as possible.

Question 24

What is the recommended radiation dose for curative treatment of Ewing Sarcoma?

Answer 24

55-60Gy total at 1.8 Gy/fraction.

Question 25

What is the recommended timing for starting EBRT in postoperative cases in Ewing Sarcoma?

Answer 25

It is recommended to start EBRT within 60 days of surgery for optimal response.

Question 26

What is the significance of shrinking the tumor before surgery?

Answer 26

Increases the likelihood of achieving clear margins with complete resection.

Question 27

What is the typical radiation dose for preoperative treatment of Ewing sarcoma?

Answer 27

Lower doses ranging from 36-45 Gy.

Question 28

What is the typical radiation dose for postoperative treatment of Ewing sarcoma?

Answer 28

Lower doses ranging from 45 to 55.8 Gy depending on the level of resection.

Question 29

What technique is recommended for radiation treatment planning of Ewing sarcoma?

Answer 29

Shrinking field technique with a 2 cm margin around the primary lesion.

Question 30

What should be spared during radiation therapy to reduce late effects?

Answer 30

Spare the non-involved epiphysis and a lateral strip of tissue to prevent lymphedema.

Question 31

What is the most important prognostic factor for Ewing sarcoma?

Answer 31

The extent of disease at the time of diagnosis.

Question 32

How does metastatic disease at diagnosis affect prognosis?

Answer 32

Patients with metastatic disease usually have a poor outcome.

Question 33

How does bone or bone marrow involvement affect prognosis?

Answer 33

Patients with bone or bone marrow involvement did not do as well as those with limited pulmonary involvement.

Question 34

What is the prognosis for patients with soft tissue extension?

Answer 34

Extension into soft tissue is associated with a less favorable prognosis than limited or no soft tissue involvement.

Question 35

What impact does tumor size have on prognosis?

Answer 35

Large tumors and those arising in the pelvis can negatively affect prognosis.

Question 36

What is the recurrence rate for Ewing sarcoma?

Answer 36

The recurrence rate is 30% to 40%, which includes local or distant disease.