Ewing’s Sarcoma

Definition
Ewing’s sarcoma is a small round-cell tumor
typically arising in the bones, rarely in soft
tissues, of children and adolescents.
 Epidemiology
Primary bone tumors account for 5% of all
cancers in childhood and Ewing sarcoma is the
second most common bone tumor in this age
group. The incidence of ESFT in the US
between 1973 and 2004 was estimated to be
approximately 3 per 1,000,000.
 Etiology
The cause of Ewing sarcoma is largely unknown.
Therefore, there is no known way of
preventing this cancer.
There is a known relationship between periods
of rapid bone growth in adolescents and the
development of primary bone cancers such as
Ewing sarcoma and osteosarcoma.
 Risk Factor
• Race/ethnicity
Ewing tumors are rare overall, but they are much more common among
whites (either non-Hispanic or Hispanic) than among Asian Americans or
African Americans. The reason for this is not known.
• Gender
These cancers are slightly more common in males than in females.
• Age
Ewing tumors can occur at any age, but they are most common in teens and
are less common among young adults and young children. They are rare in
older adults.

There are no confirmed links between any environmental factors or inherited

risk factors and the development of Ewing sarcoma. However, there are
possible factors that have been identifi ed which may increase an
individual’s risk of developing Ewing sarcoma.
Pathogenesis
Sign and Symtoms
 Diagnosis
• Medical history and physical exam
• Imaging tests : X-rays, Magnetic resonance imaging
(MRI) scan, Computed tomography (CT) scan, Bone
scan, Positron emission tomography (PET) scan
• Biopsy of the tumor: Incisional biopsy, Excisional biopsy
• Bone marrow aspiration and biopsy
• Testing biopsy samples: Immunohistochemistry,
Chromosome tests
• Blood tests
Plain films show "onion skinning"
soft tissue mass growing out from the bone giving
rise to multilamellated periosteal reaction vs
"sunburst" pattern seen in osteosarcoma.
Diaphsysis rather than metaphysis (osteosarcoma)
Periosteum displaced by underlying tumor
Codman triangle
New bone formation beyond periosteal margin
rare
Associated soft tissue mass common
 Staging
The AJCC staging system for bone cancers is based on 4 key pieces of
information:
T describes the size of the main (primary) tumor and whether it appears in
different areas of the bone.
N describes the extent of spread to nearby (regional) lymph nodes (small
beansized collections of immune system cells). Bone tumors rarely spread
to the lymph nodes.
M indicates whether the cancer has metastasized (spread) to other organs of
the body. (The most common sites of spread are to the lungs or other
bones.)
G stands for the grade of the tumor, which describes how the cells from
biopsy samples look. Low-grade tumor cells look more like normal cells
and are less likely to grow and spread quickly, while high-grade tumor cells
look more abnormal. (All Ewing tumors are considered high-grade
tumors.)
Numbers or letters after T, N, M, and G provide more details about each of
these factors.
 CHEMOTHERAPY
NEO-ADJUVANT CHEMOTHERAPY
In most cases, chemotherapy is used before surgery to kill cancer
cells and shrink the tumour to make surgery easier; this is known as
‘neo-adjuvant chemotherapy’. In the UK, Ireland, and much of
Europe, the standard chemotherapy treatment given before surgery
is made up of four drugs: Vincristine, Ifosfamide, Doxorubicin and
Etoposide (this is referred to as ‘VIDE’).
ADJUVANT CHEMOTHERAPY
Chemotherapy can also be used after surgery to destroy any
remaining cancer cells in the location of the tumour, or in the rest
of the body; this is known as ‘adjuvant chemotherapy’. In the UK,
Ireland, and much of Europe, the standard chemotherapy
treatment given after surgery is made up of four drugs: Vincristine,
Acintomycin D, Ifosfamide and Cyclophosphamide.
 SURGERY
The aim of surgery is to remove the primary
tumour to prevent its growth and spread to other areas
of the body. The surgical removal of an Ewing sarcoma
requires ‘wide-surgical margins’; this means some
healthy tissue is removed alongside the tumour to
ensure all tumour cells are removed and there is a
lower risk of the tumour returning at a later date.
The surgical procedure carried out to treat an
Ewing sarcoma is known as ‘limb-salvaging surgery’,
which aims to completely remove the tumour while
preserving as much of the normal function and
cosmetic appearance of the limb as possible.
 RADIOTHERAPY
Ewing sarcoma responds well to radiotherapy
treatment. Radiotherapy is sometimes used
instead of surgery in cases where surgical
removal of the primary tumour is not possible -
for example if the tumour is in the pelvis or spine.
Radiotherapy may also be given after surgery
to ensure all tumour cells in the area are
destroyed. The exact dose and length of
radiotherapy treatment will also be decided by a
specialist team of doctors, but it is usually given
as a single dose each day (lasting a few minutes)
for approximately 5 – 6 weeks.
 Prognosis
Disease site
• Favorable: non-pelvic
o distal, ribs and other having the best prognosis
Unfavorable: Pelvic
Intermediate: Proximal
• Age: younger is favorable
• Size: >8cm is unfavorable
• Labs
o Unfavorable: anemia, elevated ESR, leukocytosis, and
elevated LDH