Ewing Sarcoma

Description

Malignant neoplasm composed of small, undifferentiated round cells

Most common malignant bone tumour after osteosarcoma

Categories

Epidemiology

Adolescents, 15yrs

Male predilection, majority are white

Aetiopathogenesis

Originated from mesenchymal stem cell with potential of limited neural

differentiation

Translocation t(11;22) (q24;q12) which encodes EWS-FLI1 fusion protein

Common site

Long bones, pelvis and ribs

1~2% gnatic involvement (mandible > maxilla)

Clinical features

Pain, swelling

Fever, leucocytosis and elevated erythrocyte sedimentation

Tumour penetrates the cortex, resulting in soft tissue mass overlying

affected area of bone

Paresthesia, tooth mobility

Radiographic features

Non-specific

Ill-defined radiolucency (mixed radiolucent radiopaque is possible)

Characteristic “onionskin” periosteal reaction observed in long bones

Histopathology

Ewing Sarcoma 1
 Broad sheets of small, monotonous, round cells with well-delineated nuclear
outlines and ill-defined cellular borders

Variable-sized nests of tumour cells separated by fibrovascular septa,

creating lobular pattern

Extensive necrosis and haemorrhage

Investigation and Diagnosis

PAS-positive glycogen granules - non specific

Membranous immunoreactivity for CD99 (MIC2) - non specific

Chromosomal translocations by RT-PCR or fluorescence in situ hybridisation

(FISH) - more specific

Treatment

Multidrug chemotherapy with surgery and radiotheray

Differential diagnosis

Paediatric small round cell tumours: metastatic neuroblastoma, malignant

lymphoma, small cell osteosarcoma, alveolar rhabdomyosarcoma

Ewing Sarcoma 2