• Pathology: – Often involves the epiphysis of long bones – Usually around the knee – Red or brown mass with cystic degeneration
Ewing sarcoma• Malignant neoplasm of undifferentiated cells arising within the marrow cavity• Clinical features: – Males>females – Most occur in teenagers (5-20) – Presented with pain, swelling and tenderness• X-ray: – Concentric, onion skin layering of new periosteal bone
• Pathology: – Often affects the diaphysis of long bones – Most common sites are the femur, pelvis and tibia – White tan mass with necrosis and hemorrhage
Clinical Presentation• PAIN The pain may be progressive for many months, and initially be confused with more common sources such as muscle soreness, overuse injury or "growing pains." Night pain is an important clue to the true diagnosis (25%) The primary reasons for delay in the diagnosis is failure to obtain radiographs at the initial visit. Pain that fails to resolve or is present at rest or wakes the patient from sleep should alert the clinician that further evaluation is needed.
• Swelling Palpable mass is noted in up to 1/3 of patients at the first visit.• Limp In smaller children, a limp may be the only symptom• Restriction of movement of the adjacent joint• Pathological Fracture This can increase the rate of local recurrence of the tumor after surgery and decrease the patient’s overall survival• Fever, malaise or other constitutional symptoms
diagnosisLab tests-Full blood count, ESR, CRP. LDH (elevated level is associated with poor prognosis) ALP (elevated levels at diagnosis signify increased risk of pulmonary metastasis) . Platelet count Electrolyte levels Liver function tests Renal function tests Urinalysis
Imagining studiesPlain x-rays Obtain plain films of the suspected lesions in 2 views. With joint above and joint below• CT scanning CT scanning of the chest is more sensitive than is plain film radiography for assessing pulmonary metastases. MRI MRI of the primary lesion is the best method to assess the extent of intramedullary disease as well as associated soft-tissue masses .Bone Scan A bone scan should be obtained to look for skeletal metastases or multi focal disease.
• Thallium scan Monitor effects of chemotherapy Detect local recurrence of tumor• Angiography Determine vascularity of the tumor Detect vascular displacement and determine relationship of vessels to the tumor Identify vascular anomalies Estimate effects of chemotherapy.• Once all the initial imaging & lab exam has been done biopsy is performed to conform the diagnosis.
Radiology• Site• Size• Effect on bone• Response of Bone• Matrix• Cortex• Soft tissue
• Types of biopsy Fine needle aspiration Core needle biopsy Open incisional biopsy
stagingThe staging system is typically depicted as follows• Stage I: Low grade tumors I-A intra compartmental I-B extra compartmental• Stage II: High grade tumors II-A intra compartmental II-B extra compartmental• Stage III: Any tumors with evidence of metastasis
Treatment 1. Radiological staging 2. Biopsy to confirm diagnosis 3. Preoperative chemotherapy,Radiotherapy 4. Repeat radiological staging (access chemo response, finalize surgical tx plan) 5. Surgical resection with wide margin 6. Reconstruction using one of many techniques 7. Post op chemo based on preop response•
Prognostic Factors• Extant of the disease – Pts with pulmonary, non pulmonry (bone) or skip metastasis have poor prognosis• Grade of the tumor – High grade tumor have poor prognosis• Size of the primary lesion – Large size tumors have worse prognosis then small size tumors• Skeletal location – proximal tumors do worse than distal tumors.• Secondary osteosarcoma: Poor prognosis