EWING SARCOMA

Pembimbing
dr. Nunik Royyani, Sp. Rad
 MENU

introduction

pathology

Sign and symptons

Radiography

Differential
diagnose
Ewing sarcoma is a type of tumor that forms from a certain kind of cellin bone
or soft tissue. Ewing sarcoma may be found in the bones of the legs, arms, feet,
hands, chest, pelvis, spine, or skull. Ewing sarcoma also may be found in the
soft tissue of the trunk, arms, legs, head and neck, abdominal cavity, or other
areas.
Ewing sarcoma is most common in adolescents and young adults.
Ewing sarcoma has also been called peripheral primitive
neuroectodermal tumor, Askin tumor (Ewing sarcoma of the chest
wall), extraosseous Ewing sarcoma (Ewing sarcoma in tissue other than
bone), and Ewing sarcoma family of tumors.
PATHOLOGY

Ewing sarcoma is a small round blue cell tumour with regular sized primitive
appearing cells. It is closely related to the soft tissue
tumours pPNET, Askintumour and neuroepithelioma, which collectively are
referred to as Ewing sarcoma family of tumours (ESFT) 1. They share not
only microscopic appearances but also demonstrate a non-random
t(11;22)(q24;q12) chromosome rearrangement.
SIGN AND SYMPTOMS
 Localized pain
 Back pain, which may indicate a paraspinal, retroperitoneal, or
deep pelvic tumor
 Palpable mass
 Systemic symptoms of fever and weight loss, which often indicate
metastatic disease
 Anemia
 Leukocytosis
 Elevated erythrocyte sedimentation rate
PLAIN RADIOGRAPHY
Presents as extensive diaphyseal lesion

At midshaft of the long bone, the cortex displays increased

density, extending externally as periosteal new bone, forming
multiple thin parallel layers giving onion peel appearence
ANGIOGRAM

• Shows hypervascular reaction & intrinsic

neoplastic vasculature quite well
• Early arterial phase shows the reactive
and a capsular vessel as well as extent of
the soft tissue mass
CT SCAN

- Shows detail of
radiolucent portion
of the lesion and
areas of cortical
destruction
- Does not outline
the soft tissue
extent
MRI

• Most precise in defining

the local extent of the
tumor
• Evaluate the extent of
tissue masses
• For staging and surgical
planning
• To assesses the response
to neoadjuvant
chemotherapy or
irradiation
ISOTOPS SCANS
- Technetium-99 whole body
radionucleotide bone scan to
identify skeletal metastasis
- Fluorine-18
fluorodeoxyglucose positron
emission tomography (FDG-
PET) increases the sensitivity
of detection of metastasis
- Will show other clinically
unsuspected sites
DIFFERENTIAL DIAGNOSIS
• Chronic osteomyelitis
•Metastatic neuroblastoma – first 3 years of life, neural
filamentous material, elevated urinary catecholamines,
homer-wright rosettes
•Lymphosarcoma – larger nuclei and less uniform, lack of
intracellular glycogen
•Reticulum cell sarcoma – PAS negative and reticulin
positive (doesn’t have glycogen)
Ewing sarcoma Chronic Osteomyelitis

Most common in diaphysis Most common in metaphysis

Very aggressive Not aggressive

Moth eaten and osteoporotic and

Onion peel
areas of sclerotic

More of small round cell Shows granulocytes

Soft tissues mass commonly

Ulceration and sinuses of the skin
accompanies the lesion
TREATMENT
Multiagent chemotherapy
 Most protocols administer preoperative chemotherapy then surgery is
performed. Surgery is followed by several courses of postoperative
chemotherapy.
 Ewing sarcoma responds well to chemotherapy. Often there is a dramatic
reduction in size of the tumor.

Surgical resection
 Limb sparing surgery whenever feasible unless there will be a large leg
length discrepancy that can not be accomodated for with surgery
 Rarely ever an amputation since Ewing sarcoma are sensitive to radiation
If surgical resection is not feasible, radiation may be utilized for local control
(instead of an amputation) since Ewing sarcoma is highly sensitive to radiation
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