Ewing sarcoma

Ewing sarcoma

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  EWING’S SARCOMA PRITHWIRAJ MAITI FINALYEAR MBBS R.G.KAR MEDICAL COLLEGE 5.3.2014
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  Introduction • It isthe 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.
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  • Location affected:Diaphysis of long bone. • Common bones involved: (in decreasing order) 1. Femur, 2. Tibia, 3. Fibula, 4. Humerus, 5. Flat bones.
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  Clinical features • • • • • • Throbbing, intermittentpain. Pain worst at night. Red and warm skin. Swelling over diaphyseal region. Generalized illness and pyrexia. Mimics acute osteomyelitis.
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  Pathology • Lobulated largemass 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.
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  Histology • • • • • • Highly cellular. Little intercellularmatrix. Necrosis is common. Cells are arranged around the vessels. PAS +Ve (due to presence of glycogen). HBA71 +Ve (it is an immunological marker).
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  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).
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  • 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.
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  • Bone scan: Detectionof the extent of tumour involvement. Detection of the skip lesions in the same bone. Detection of distant metastasis.
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  Course • Repeated episodesof 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.
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  Management of Ewing’ssarcoma 1. Radiotherapy, 2. Surgery, 3. Chemotherapy.
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  Radiotherapy • Ewing’s sarcomais 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.
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  Surgery • Surgery hasa very little role in the management of Ewing sarcoma. • The available options are: 1. Debulking surgery. 2. Limb preservation surgery.
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  Systemic Chemotherapy • Itis 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.
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  Comment Best result canbe achieved only by combining all the 3 options available.
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  Prognostic Factors • • • • • • • • Site: Humerusand pelvis-> Bad prognosis. Stage: Metastasis-> Bad prognosis. Tumour size. Response to chemotherapy. Male gender. High LDH level. Anemia. c-MIC/ ki-67 gene expression.