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