EWING’S SARCOMA

PRITHWIRAJ MAITIFINAL YEAR MBBS

R.G.KAR MEDICAL COLLEGE5.3.2014

Introduction

• It is the 3rd most common primary malignant bone tumor.

• Arises from endothelial cells of bone marrow.• Age: First 2 decades of life.• Common in males.• Uncommon in blacks.

• Location affected: Diaphysis of long bone.• Common bones involved: (in decreasing order)1. Femur,2. Tibia,3. Fibula,4. Humerus,5. Flat bones.

Clinical features

• Throbbing, intermittent pain.• Pain worst at night.• Red and warm skin.• Swelling over diaphyseal region.• Generalized illness and pyrexia.• Mimics acute osteomyelitis.

Pathology

• Lobulated large mass over diaphysis of long bone.• When hemorrhage occurs, it appears like a red

current jelly.• Extensive medulary invasion and destruction of

endosteum and cortex.• Prominent peiosteal new bone formation.• Sometimes, the tumor is liquefied so as to

resemble a pus.

Histology

• Highly cellular.• Little intercellular matrix.• Necrosis is common.• Cells are arranged around the vessels.• PAS +Ve (due to presence of glycogen).• HBA71 +Ve (it is an immunological marker).

Investigations• Blood: Anemia, Leucocytosis, Raised ESR, Raised LDH.• X-Ray: Diaphyseal lesion with irregular destruction (Moth eaten

appearance). Periosteal new bone formation (Onion peel appearance). X-Ray chest (to detect metastasis).

• CT Scan:Detect the extent of cortical destruction.CT scan chest: Detection of metastasis.• MRI:Detection of the degree of intraosseus and

extraosseus extension of tumor. Initial staging and surgical planning.Assessment of response of the tumor to

chemotherapeutic regimen.

• Bone scan:Detection of the extent of tumour

involvement.Detection of the skip lesions in the same

bone.Detection of distant metastasis.

Course

• Repeated episodes of exacerbation and remission is characteristic.

• Metastasize by hematogenous and lymphatogenous routes.

• It obeys the course of bone to bone metastasis (another one example is osteosarcoma).

• Common bones where Ewing’s sarcoma metastasizes are skull, vertebra, rib and lung.

Management of Ewing’s sarcoma

1. Radiotherapy,2. Surgery,3. Chemotherapy.

Radiotherapy

• Ewing’s sarcoma is highly radiosensitive tumour.

• Radiotherapy has dramatic effect on Ewing’s sarcoma (Melts like snow).

• But recurrence rates are high in case of radiotherapy alone, survival rates are also not satisfactory.

Surgery

• Surgery has a very little role in the management of Ewing sarcoma.

• The available options are:1. Debulking surgery.2. Limb preservation surgery.

Systemic Chemotherapy

• It is the mainstay of treatment of Ewing’s sarcoma.

• Response to chemotherapy is the most important prognostic factor in this disease.

• Common agents used are: Ifosfamide/ Actinomycin D/ Doxorubicin/ Vincristine etc.

Comment

Best result can be achieved only by combining all the 3 options available.

Prognostic Factors

• Site: Humerus and pelvis-> Bad prognosis.• Stage: Metastasis-> Bad prognosis.• Tumour size.• Response to chemotherapy.• Male gender.• High LDH level.• Anemia.• c-MIC/ ki-67 gene expression.