CEWING’S

SARCOMABy Dr. Afiqi Fikri

Case Scenario•An 11 year old boy presents with h/o pain and diffuse bony swelling over the mid shaft of his right fibula since last one month. •The mother gives h/o intermittent fever and redness at the site of pain. •He also has LOW and LOA.

Differential Diagnosis•Ewing’s tumour•Osteosarcoma•Osteomyelitis•Osteoid osteoma

Diagnosis Points favouring Points against

Ewing’s tumour Age 5-15 Site: Diaphysis Femur, tibia, flat bone Pain, Swelling, often fever for weeks-monthsLOW, LOA

Osteosarcoma Pain, swelling Femur, tibia LOW, LOA

Metaphysis Age:15-25 years

Osteomyelitis Pain, swelling, redness, fever can have LOA, LOW

No sequestrum, cloacae Usually at metaphysis No h/o trauma

Osteoid osteoma Commonest benign Age: 5-25 Common in LL (tibia) Pain +- swelling

Fever Redness LOW, LOA

Investigations• Laboratory:

FBC (Hb, Hct, TWBC) Serum ALP

• ImagingX-ray right tibia and fibula (AP and lateral view) CT/MRI Radioisotope bone scan

• Biopsy & HPE

Clinical Features of Ewing’s• Highly malignant tumour• Age: 10-20 • Bones: Long bones (femur, tibia), flat bones (pelvis, calcaneum), multicentric origin• Site: Diaphysis• Pain + Swelling• Often associated with fever• Highly radio sensitive• Very poor prognosis (5 year survival rate 30%)

X-Ray findings• Usually show an area of bone destruction which, unlike osteosarcoma, is predominantly in the mid-diaphysis.• New bone formation may extend along the shaft and sometimes appears as fusiform layers of bone around the lesion – ‘onion-peel’ effect.• Often the tumour extends into the surrounding tissues, with radiating streaks of ossification and reactive periosteal bone at the proximal and distal margins. • The ‘sunray’ appearance and Codman’s triangle.

Principles of Treatment• Highly radio-sensitive tumour which melts quickly but recurs.• Distant metastasis is fast• To control local tumour by radiotherapy (6000 rads)• To control metastasis by chemotherapy

Vincristine Cyclophosphamide Adriamycin

• Repeat every 3-4 weeks for 12-18 cycles.

Best results;•A course of preoperative neo-adjuvant chemotherapy•Then wide excision if tumor is in a favorable site

OR if less accessible;•Radiotherapy followed by local excision•Then further chemotherapy course for 1 year

Principles of Treatment