EWING'S SARCOMA DIAGNOSIS AND TREATMENT

 

 

Introduction :

 

Ewing's Sarcoma-Highly malignant Tumour, believed to arise from endothelial

 cells of bone marrow,

 

Dr. James Ewing who first described it in 1921.

 

Ewing's sarcoma is characterized by aggressive growth and tends to spread to

other parts of the body.

 

Exact cause is unknown, but it's believed to occur with gene alteration.

 

 

Clinical Features:

 Age-10-20y,

  Site-Tubular bone-Femur,Tibia   Fibula,Clavicle

  Pain-Thrombing in nature,

  Swelling, Pyrexia,

  Tender,

  Warm,

  Palpable swelling,

 

 

Investigation

Laboratory Findings-

Imaging

Histopathology

 

•Laboratory Findings-

•ESR-Raised, Hb% Decreased

•Imaging-

•X-ray

•CT, MRI,

•Bone Scan

 

X-ray :

• Commonly involve diaphysis,

 

• Area of bone destruction

 

• Onion peel effect

 New bone formation-along the shaft -as a fusiform layer around the lesion.

 

• Sometimes extends into soft tissue,(as Osteosarcoma)

 

CT Scan-

• Extends of tumour

• Cortical destruction and a soft tissue mass

• Onion-skinning or a Codman's triangle

• CT Scan of Lung for metastasis.

MRI-

 

• Tumor Mass

• Bone Involvement

• Soft Tissue Extension

• Periosteal Reaction

• Enhancement Pattern

Bone Scan -

  • Shows-areas of activity

 

Pathology-

 

Macroscopic-Lobulated,and large, Grey(brain) or red redcurrent jelly if

 hemorrhage

Microscopic-Small Dark polyhedral cells with no regular arrangement.

 And no ground substance

 

 

Diagnosis:

• Bone infection,

• Other round cell tumour- 

         Reticulum cell sarcoma,

          Metastatic neuroblastoma.

 

Treatment:

 

• Combination of chemotherapy, Radiotherapy, with surgery.

• A course of neo adjuvant chemotherapy, then wide excision if favorable site.

• Or Radiotherapy followed by Local excision if less accessible,

• Then a further course of chemotherapy for 1y

• Post operative Radiotherapy added if specimen found not to have sufficiently

  wide margin of normal tissue.

 

 

Prognosis- 

 5 years survival rate- about 50%, Up to 70% with no metastatic Ewing's Sarcoma.