Ewing Sarcoma Flashcards

1
Q
A
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2
Q

What is a sarcoma?

A

A malignant tumour that arises from connective tissue origin.

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3
Q

Who is Dr. James Ewing?

A

An individual associated with the study of Ewing’s sarcoma, which is the most common bone tumour in pediatrics.

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4
Q

What percentage of childhood cancers does Ewing’s sarcoma account for?

A

1%.

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5
Q

What is the most common site for Ewing’s sarcoma?

A

The femur.

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6
Q

What are ‘Small Blue Round Cells’?

A

Cells that lack specific markers for lymphoma, neuroblastoma, and rhabdomyosarcoma, with a diffuse pattern being the most common.

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7
Q

What age range does Ewing’s sarcoma typically affect?

A

5 months to 60 years, peaking at 10-20 years of age.

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8
Q

True or False: Ewing’s sarcoma is commonly inherited.

A

False.

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9
Q

What is the known cause of Ewing’s sarcoma?

A

Relatively unknown; not inherited and no identifiable risks.

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10
Q

What type of chromosomal abnormality is commonly associated with Ewing’s sarcoma?

A

Fusion transcript involving chromosome rearrangement between genes on Chromosome 11 and 22.

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11
Q

In which bones does Ewing’s sarcoma most often occur?

A

Extremities, particularly in the long bones.

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12
Q

What are common symptoms of Ewing’s sarcoma?

A

Pain, swelling, redness, palpable mass, pathological fracture, pyrexia, weight loss.

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13
Q

What imaging techniques are important for determining the extent of Ewing’s sarcoma?

A

CT, MRI, PET/CT, Bone Scan.

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14
Q

What is a ‘Skip Metastasis’?

A

A secondary tumour separate from the primary tumour, occurring in the same bone or adjacent joint.

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15
Q

What is the significance of tumour grade in Ewing’s sarcoma?

A

It is a significant prognostic factor, with grades classified as G1 (low), G2 (high), G3 (Ewing’s).

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16
Q

What does the AJCC staging system incorporate?

A

Tumour size, grade, and histology.

17
Q

What is the 5-year survival rate for Ewing’s sarcoma?

A

Roughly 70%.

18
Q

What was the most common treatment option for Ewing’s sarcoma historically?

A

Amputation.

19
Q

What is the standard treatment approach for localized Ewing’s sarcoma?

A

Multidisciplinary approach including surgery and radiation therapy.

20
Q

What is induction chemotherapy?

A

Chemotherapy given before surgery to evaluate effectiveness and shrink the tumour.

21
Q

What factors determine the type of surgery for Ewing’s sarcoma?

A

Tumour location, age, other treatments used, extent of disease.

22
Q

What is the treatment modality of choice for unresectable tumours?

A

Radiation therapy.

23
Q

What is the dose range for pre-operative radiation therapy in Ewing’s sarcoma?

A

3600 – 4500 cGy.

24
Q

What is the total dose limit historically for radiation therapy due to secondary malignancies?

A

5580 cGy.

25
Q

What is the common recurrence rate for Ewing’s sarcoma?

A

30-40%.