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Ewing Sarcoma

Ewing sarcoma is a highly malignant cancer that peaks in adolescents and young adults. It arises in bone or soft tissue and is the second most common malignant bone tumor in children and young adults. A translocation of the EWSR1 gene with an ETS transcription factor gene occurs in over 95% of Ewing sarcomas. Treatment involves multi-agent chemotherapy for at least 12 weeks followed by local therapy and possible additional chemotherapy depending on the stage of cancer.

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88 views12 pages

Ewing Sarcoma

Ewing sarcoma is a highly malignant cancer that peaks in adolescents and young adults. It arises in bone or soft tissue and is the second most common malignant bone tumor in children and young adults. A translocation of the EWSR1 gene with an ETS transcription factor gene occurs in over 95% of Ewing sarcomas. Treatment involves multi-agent chemotherapy for at least 12 weeks followed by local therapy and possible additional chemotherapy depending on the stage of cancer.

Uploaded by

Vany Maksum
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Ewing sarcoma

Definition
• Ewing sarcoma family of tumors (ESFT) form a group of highly
malignant diseases that peak in incidence in adolescence and early
adult life. These tumors arise in either bone or soft tissue .
Epidemiology
The second most common malignant bone tumour occurring in
children and young adults, and accounts for 10–15% of all primary
bone tumours.1 The annual incidence is approximately 0.6/million total
population, and it usually occurs between the ages of 10 and 20 years.
It affects 13/million 0–24 year olds each year in the UK,2 and is slightly
more common in males than females (ratio, 1.5 : 1)
Etiology
• Translocation of EWSR1 (Ewing sarcoma breakpoint region 1) with
an ETS (E26 transformation-specific) transcription factor gene occurs
in more than 95% of Ewing sarcomas
• he most common translocation seen in about 85% of all Ewing tumor
is the t(11;22) translocation. This translocation joins the Ewing
sarcoma gene EWS on chromosome 22 to a gene of the ETS family
Risk factor
1. Parental exposure to pesticides, solvents chemistry as well as work in
the agriculture fields
2. Hernias, both inguinal and umbilical /
3. Increasing the age of the mother and father as they occur
conception
Pathogenesis
• More than 85% of Ewing's sarcomas are related to translocation of
the t chromosome (11; 22) (q24; q12) which results
• the EWS-FLI-1 combined gene. The protein produced is an aberrant
transcription factor that deregulates the target cell's gene expression
program, thus revealing a primitive neuroectodermal phenotype
Clinical features
- Lesions on skeletal tend to develop into a tumor large and integrated
with soft tissue in a few weeks.
- The initial symptom that is often found is mild pain that disappears
but will develop very quickly into severe pain and requires analgesics
- Tumor growth will cause edema and inflammation at the location of
the lesion.
- Other symptoms that are often found include fever, anemia,
decreased appetite and weight gain
- Laboratory
Increased LDH, increased ESR, Leukocytosis.
- Radiology
*onion skin appearance (diaphysis of long bone)
• Sunburst
• Osteolysis
• Periosteal reaction
• Biopsy
- Small round cells
- The cytoplasmic
characteristics are
pale, vacuol with
indefinite
boundaries.
- In contrast to the
cytoplasm, the
picture of the ES
nucleus is clear with
a clear color.
Treatment
**NCCN recommendations for treatment of Ewing sarcoma are as
follows :
• Enrollment in a clinical trial should be considered when available; in
addition, whenever possible patients should be referred to a tertiary
care center with expertise in sarcoma, for treatment by a
multidisciplinary team
• Multi-agent chemotherapy for at least 12 weeks followed by local
control therapy and adjuvant treatment; longer duration of initial
chemotherapy can be considered for patients with metastatic disease,
based on response
• VAC/IE (vincristine, doxorubicin [Adriamycin], and cyclophosphamide
alternating with ifosfamide and etoposide) is the preferred regimen
for localized disease
• VAC (vincristine, doxorubicin, and cyclophosphamide) is the preferred
regimen for patients with metastatic disease
• Restaging following chemotherapy

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